Diagnosing headache type (beta)

B. Focal neurological deficit of ischaemic originlasting <24 hoursC. Headache develops simultaneously with onset offocal deficitD. Headache resolves within 24 hoursDiagnosing headache type comment:Whilst more common with basilar- than carotidterritoryTIA, headache is very rarely a prominentsymptom of TIA. The differential diagnosis betweenTIA with headache and an attack of migraine withaura may be particularly difficult. The mode of onsetis crucial: the focal deficit is typically sudden in aTIA and more frequently progressive in a migrainousaura. Furthermore, positive phenomena (eg,scintillating scotoma) are far more common inmigrainous aura than in TIA whereas negative phenomenaare more usual in TIA.6.2 Headache attributed to non-traumatic intracranialhaemorrhageCoded elsewhere:Headache attributed to traumatic intracerebraland/or subarachnoid haemorrhage or to traumaticintracerebral haematoma is coded as 5.1.1 Acute posttraumaticheadache attributed to moderate or severe headinjury or 5.2.1 Chronic post-traumatic headache attributedto moderate or severe head injury.Headache attributed to traumatic epiduralhaematoma is coded as 5.5.1 Headache attributed toepidural haematoma; headache attributed to traumaticsubdural haematoma is coded as 5.5.2 Headacheattributed to subdural haematoma.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 676.2.1 Headache attributed to intracerebralhaemorrhageDiagnosing headache type diagnostic criteria:A. Any new acute headache fulfilling criterion CB. Neurological signs or neuroimaging evidence ofrecent non-traumatic intracerebral haemorrhageC. Headache develops simultaneously with or invery close temporal relation to intracerebralhaemorrhageDiagnosing headache type comments:Through usage, the term intracerebral is taken in thiscontext to include intracerebellar.Headache is more common and more severe inhaemorrhagic than in ischaemic stroke. It is usuallyovershadowed by focal deficits or coma, but it canbe the prominent early feature of cerebellar haemorrhagewhich may require emergency surgicaldecompression.6.2.1 Headache attributed to intracerebral haemorrhageis more often due to associated subarachnoid bloodand to local compression than to intracranial hypertension.It can occasionally present as thunderclapheadache.6.2.2 Headache attributed to subarachnoidhaemorrhage (SAH)Diagnosing headache type diagnostic criteria:A. Severe headache of sudden onset fulfilling criteriaC and DB. Neuroimaging (CT or MRI T2 or flair) or CSFevidence of non-traumatic subarachnoid haemorrhagewith or without other clinical signsC. Headache develops simultaneously withhaemorrhageD. Headache resolves within 1 monthDiagnosing headache type comments:Subarachnoid haemorrhage is by far the mostcommon cause of intense and incapacitatingheadache of abrupt onset (thunderclap headache)and remains a serious condition (50% of patients diefollowing SAH, often before arriving at hospital, and50% of survivors are left disabled).Excluding trauma, 80% of cases result from rupturedsaccular aneurysms.The headache of SAH is often unilateral at onsetand accompanied by nausea, vomiting, disorders ofconsciousness and nuchal rigidity and less frequentlyby fever and cardiac dysrythmia. However,it may be less severe and without associated signs.The abrupt onset is the key feature. Any patient withheadache of abrupt onset or thunderclap headacheshould be evaluated for SAH. Diagnosis is confirmedby CT scan without contrast or MRI (flairsequences) which have a sensitivity of over 90% inthe first 24 hours. If neuroimaging is negative,equivocal or technically inadequate, a lumbar punctureshould be performed.Subarachnoid haemorrhage is a neurosurgicalemergency.6.3 Headache attributed to unruptured vascularmalformationCoded elsewhere:Headache attributed to ruptured vascular malformationis coded as 6.2.1 Headache attributed to intracerebralhaemorrhage or 6.2.2 Headache attributed tosubarachnoid haemorrhage.6.3.1 Headache attributed to saccular aneurysmDiagnosing headache type diagnostic criteria:A. Any new acute headache including thunderclapheadache and/or painful third nerve palsy fulfillingcriteria C and DB. Neuroimaging evidence of saccular aneurysmC. Evidence exists of causation by the saccularaneurysmD. Headache resolves within 72 hoursE. Subarachnoid haemorrhage, intracerebral haemorrhageand other causes of headache ruled outby appropriate investigationsDiagnosing headache type comments:Headache is reported by approximately 18% ofpatients with unruptured cerebral aneurysm.It usually has no specific features. However, thunderclapheadache occurs prior to confirmed aneurysmalSAH in about 50% of patients. Althoughthunderclap headache may occur in the absence ofvascular malformations, such malformations shouldbe looked for by appropriate non-invasive investigations(MRA or CT angiography) and, in doubtfulcases, by conventional angiography. Aclassic varietyof ‘warning pain’ (signalling impending rupture orprogressive enlargement) is an acute third nervepalsy with retro-orbital pain and a dilated pupil,indicating an aneurysm of the posterior communicatingcerebral artery or end of carotid artery.Diagnosing headache type (IHS 2003)68 ICHD-II6.3.2 Headache attributed to arteriovenousmalformation (AVM)Diagnosing headache type diagnostic criteria:A. Any new acute headache fulfilling criteria C andD

B. Neuroimaging evidence of arteriovenousmalformationC. Evidence exists of causation by the arteriovenousmalformationD. Headache resolves within 72 hoursE. Subarachnoid haemorrhage, intracerebral haemorrhageand other causes of headache ruled outby appropriate investigationsDiagnosing headache type comments:Cases have been reported highlighting the associationof AVM with a variety of headaches such ascluster headache, chronic paroxysmal hemicrania(CPH) and short-lasting unilateral neuralgiformheadache with conjunctival injection and tearing(SUNCT), but these cases had atypical features. Thereis no good evidence of a relationship between AVMand these primary headaches when they are typical.Migraine with aura has been reported in up to 58%of women with AVM. A strong argument in favourof a causal relationship is the overwhelming correlationbetween the side of the headache or of theaura and the side of the AVM. There is thus a strongsuggestion that AVM can cause attacks of migrainewith aura (symptomatic migraine). Yet in large AVMseries, migraine as a presenting symptom is rare,much less common than haemorrhage, epilepsy orfocal deficits.6.3.3 Headache attributed to dural arterio-venousfistulaDiagnosing headache type diagnostic criteria:A. Any new acute headache fulfilling criterion CB. Neuroimaging evidence of dural arteriovenousfistulaC. Evidence exists of causation by the fistulaD. Subarachnoid haemorrhage, intracerebral haemorrhageand other causes of headache ruled outby appropriate investigationsDiagnosing headache type comment:Studies devoted to headache with dural arteriovenousfistula are lacking. A painful pulsatile tinnituscan be a presenting symptom, as well as headachewith other signs of intracranial hypertension due todecrease in venous outflow and sometimes to sinusthrombosis. Carotido-cavernous fistulae may presentas painful ophthalmoplegia.6.3.4 Headache attributed to cavernous angiomaCoded elsewhere:Headache attributed to cerebral haemorrhage orseizure secondary to cavernous angioma is coded as6.2.1 Headache attributed to intracerebral haemorrhage or7.6 Headache attributed to epileptic seizure.Diagnosing headache type diagnostic criteria:A. Any new acute headache fulfilling criterion CB. Neuroimaging evidence of cavernous angiomaC. Evidence exists of causation by the cavernousangiomaD. Subarachnoid haemorrhage, intracerebral haemorrhageand other causes of headache ruled outby appropriate investigationsDiagnosing headache type comment:Cavernous angiomas are increasingly recognisedon MRI. There is no good study devoted toheadache associated with these malformations.Headache is commonly reported as a consequenceof cerebral haemorrhage or of seizures due tocavernous angioma and should be coded to theseaccordingly.6.3.5 Headache attributed to encephalotrigeminalor leptomeningeal angiomatosis (Sturge Webersyndrome)Diagnosing headache type diagnostic criteria:A. Any new acute headache fulfilling criterion CB. Facial angioma, seizures and neuroimaging evidenceof meningeal angioma ipsilateral to thefacial angiomaC. Evidence exists of causation by the angiomasD. Other causes of headache ruled out by appropriateinvestigationsDiagnosing headache type comment:Headache is commonly reported in this conditionbut poorly documented. Isolated cases suggest thatencephalotrigeminal or leptomeningeal angiomatosismay be a cause of symptomatic migraine, particularlyof attacks with prolonged auras (possiblyrelated to chronic oligaemia).6.4 Headache attributed to arteritis6.4.1 Headache attributed to giant cell arteritis(GCA)Diagnosing headache type previously used terms:Temporal arteritis, Horton’s diseaseDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 69Diagnosing headache type diagnostic criteria:A. Any new persisting headache fulfilling criteria Cand DB. At least one of the following:1. swollen tender scalp artery with elevated erythrocytesedimentation rate (ESR) and/or Creactive protein (CRP)2. temporal artery biopsy demonstrating giantcell arteritisC. Headache develops in close temporal relation toother symptoms and signs of giant cell arteritisD. Headache resolves or greatly improves within 3days of high-dose steroid treatmentDiagnosing headache type comments:Of all arteritides and collagen vascular diseases,giant cell arteritis is the disease most conspicuouslyassociated with headache (which is due to inflammationof head arteries, mostly branches of the externalcarotid artery). The following points should bestressed:• the variability in the characteristics of headacheand other associated symptoms of GCA(polymyalgia rheumatica, jaw claudication) aresuch that any recent persisting headache in apatient over 60 years of age should suggest GCAand lead to appropriate investigations;• recent repeated attacks of amaurosis fugax associatedwith headache are very suggestive of GCAand should prompt urgent investigations;• the major risk is of blindness due to anteriorischaemic optic neuropathy, which can be preventedby immediate steroid treatment;• the time interval between visual loss in one eyeand in the other is usually less than 1 week;• there are also risks of cerebral ischaemic eventsand of dementia;• on histological examination, the temporal arterymay appear uninvolved in some areas (skiplesions) pointing to the necessity of serialsectioning;• duplex scanning of the temporal arteries mayvisualise the thickened arterial wall (as a halo onaxial sections) and may help to select the site forbiopsy.6.4.2 Headache attributed to primary centralnervous system (CNS) angiitisDiagnosing headache type previously used terms:Isolated CNS angiitis, granulomatous CNS angiitisDiagnosing headache type diagnostic criteria:A. Any new persisting headache fulfilling criteria Dand EB. Encephalic signs of any type (eg, stroke, seizures,disorders of cognition or consciousness)C. CNS angiitis proven by cerebral or meningealbiopsy or suspected on angiographic signs in theabsence of systemic arteritisD. Headache develops in close temporal relation toencephalic signsE. Headache improves within 1 month of steroidand/or immunosuppressive treatmentDiagnosing headache type comments:Headache is the dominant symptom in CNS angiitis(either primary or secondary). It is present in 50–80%of cases according to the diagnostic methods used,respectively angiography and histology. Neverthelessit has no specific features and is therefore of littlediagnostic value until other signs are present such asfocal deficits, seizures, altered cognition or disordersof consciousness. However, the absence of bothheadache and CSF pleocytosis makes CNS angiitisunlikely.The pathogenesis of the headache is multifactorial:inflammation, stroke (ischaemic or haemorrhagic),raised intracranial pressure and/or SAH.The effect of treatment is far less dramatic than in6.4.1 Headache attributed to giant cell arteritis. Histologicallyproven primary CNS angiitis remains aserious and not infrequently lethal condition.6.4.3 Headache attributed to secondary centralnervous system (CNS) angiitisDiagnosing headache type diagnostic criteria:A. Any new persisting headache fulfilling criteria Dand EB. Encephalic signs of any type (eg, stroke, seizures,disorders of cognition or consciousness)C. Evidence of systemic arteritisD. Headache develops in close temporal relation toencephalic signsE. Headache improves within 1 month of steroidand/or immunosuppressive treatmentDiagnosing headache type comments:Headache is the dominant symptom in CNS angiitis(either primary or secondary). It is present in 50–80%of cases according to the diagnostic methods used,respectively angiography and histology. Neverthelessit has no specific features and is therefore of littlediagnostic value until other signs are present such asfocal deficits, seizures, altered cognition or disordersof consciousness. However, the absence of bothheadache and CSF pleocytosis makes CNS angiitisunlikely.Diagnosing headache type (IHS 2003)70 ICHD-IIThe difficulty here is two-fold: 1) diagnosing CNSangiitis in a patient known to have one of the manyconditions that can cause angiitis; 2) finding theunderlying condition (inflammatory, infectious,malignant, toxic) in a patient presenting with CNSangiitis.The pathogenesis of the headache is multifactorial:inflammation, stroke (ischaemic or haemorrhagic),raised intracranial pressure and/orsubarachnoid haemorrhage.6.5 Carotid or vertebral artery pain6.5.1 Headache or facial or neck pain attributed toarterial dissectionDiagnosing headache type diagnostic criteria:A. Any new headache, facial pain or neck pain ofacute onset, with or without other neurologicalsymptoms or signs and fulfilling criteria C and DB. Dissection demonstrated by appropriate vascularand/or neuroimaging investigationsC. Pain develops in close temporal relation to andon the same side as the dissectionD. Pain resolves within 1 monthDiagnosing headache type comments:Headache with or without neck pain can be the onlymanifestation of cervical artery dissection. It is by farthe most frequent symptom (55–100% of cases) andit is also the most frequent inaugural symptom(33–86% of cases).Headache and facial and neck pain are usuallyunilateral (ipsilateral to the dissected artery), severeand persistent (for a mean of 4 days). However, it hasno constant specific pattern and it can sometimes bevery misleading, mimicking other headaches suchas migraine, cluster headache, primary thunderclapheadache and SAH (particularly since intracranialvertebral artery dissection can itself present withSAH). Associated signs are frequent: signs of cerebralor retinal ischaemia and local signs. A painfulHorner’s syndrome or a painful tinnitus of suddenonset are highly suggestive of carotid dissection.Headache usually precedes the onset of ischaemicsigns and therefore requires early diagnosis andtreatment. Diagnosis is based on Duplex scanning,MRI, MRA and/or helical CT and, in doubtful cases,conventional angiography. Several of these investigationsare commonly needed since any of them canbe normal. There have been no randomised trialsof treatment but there is a consensus in favour ofheparin followed by warfarin for 3–6 months accordingto the quality of the arterial recovery.6.5.2 Post-endarterectomy headacheDiagnosing headache type diagnostic criteria:A. Acute headache with one of the following sets ofcharacteristics and fulfilling criteria C and D:1. diffuse mild pain2. unilateral cluster-like pain occurring once ortwice a day in attacks lasting 2–3 hours3. unilateral pulsating severe painB. Carotid endarterectomy has been performedC. Headache, in the absence of dissection, developswithin 1 week of surgeryD. Headache resolves within 1 month after surgeryDiagnosing headache type comment:Three subforms of headache have been describedafter carotid endarterectomy. The most frequent (upto 60% of cases) is a diffuse, mild isolated headacheoccurring in the first few days after surgery. It isa benign self-limiting condition. The second type(reported in up to 38% of cases) is a unilateralcluster-like pain with attacks, lasting 2–3 hours,occurring once or twice a day. It resolves in about 2weeks. The third type is part of the rare hyperperfusionsyndrome with a unilateral pulsating andsevere pain occurring after an interval of 3 days aftersurgery. It often precedes a rise in blood pressureand the onset of seizures or neurological deficits onabout the 7th day. Urgent treatment is required sincethese symptoms can herald cerebral haemorrhage.6.5.3 Carotid angioplasty headacheDiagnosing headache type diagnostic criteria:A. Any new acute headache fulfilling criteria C andD

B. Extra- or intracranial angioplasty has beenperformedC. Headache, in the absence of dissection, developsduring or within 1 week of angioplastyD. Headache resolves within 1 monthDiagnosing headache type comments:Percutaneous transluminal angioplasty (PTA) andstenting versus surgery are presently undergoingrandomised trials. Data on headache are still scarceand headache is not mentioned in large series ofcarotid PTA. In a small series of 53 patients, cervicalpain occurred in 51% of patients and head pain in33% during balloon inflation. It mostly disappearedwithin seconds of balloon deflation.Headache as part of a hyperperfusion syndrome(see 6.5.2 Post-endarterectomy headache) has also beenreported after carotid PTA.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 716.5.4 Headache attributed to intracranialendovascular proceduresDiagnosing headache type diagnostic criteria:A. Unilateral severe localised headache of abruptonset and fulfilling criteria C and DB. Intracranial angioplasty or embolisation has beenperformedC. Headache develops within seconds of theprocedureD. Headache resolves within 24 hours after the endof the procedureDiagnosing headache type comment:A very specific subform of headache has beenreported after balloon inflation or embolisation of anAVM or aneurysm. It is a severe pain of abrupt onset,localised in specific areas according to the arteryinvolved, occurring within a few seconds of the procedureand disappearing rapidly.6.5.5 Angiography headacheDiagnosing headache type diagnostic criteria:A. Acute headache with one of the following sets ofcharacteristics and fulfilling criteria C and D1. diffuse burning severe headache2. headache, in a patient with migraine, havingthe features of migraineB. Intra-arterial carotid or vertebral angiographyhas been performedC. Headache develops during angiographyD. Headache resolves within 72 hoursDiagnosing headache type comment:The intracarotid or intravertebral injection of contrastinduces a diffuse severe headache with aburning sensation which resolves spontaneously.The injection can also trigger a migraine attack in aperson who has migraine. This should be coded bothunder 1. Migraine (as the appropriate subtype) andas 6.5.5 Angiography headache.6.6 Headache attributed to cerebral venous thrombosis(CVT)Diagnosing headache type diagnostic criteria:A. Any new headache, with or without neurologicalsigns, fulfilling criteria C and DB. Neuroimaging evidence of cerebral venousthrombosisC. Headache (and neurological signs if present)develops in close temporal relation to CVTD. Headache resolves within 1 month after appropriatetreatmentDiagnosing headache type comments:Headache is by far the most frequent symptom ofCVT (present in 80–90% of cases) and it is also themost frequent inaugural symptom. It has no specificcharacteristics. Most often it is diffuse, progressive,severe and associated with other signs of intracranialhypertension. It can also be unilateral and sudden,and sometimes very misleading, mimickingmigraine, primary thunderclap headache, CSFhypotension or SAH (of which it can be a cause).Headache can be the only manifestation of CVT butin over 90% of cases it is associated with focal signs(neurological deficits or seizures) and/or signs ofintracranial hypertension, subacute encephalopathyor cavernous sinus syndrome.Given the absence of specific characteristics, anyrecent persisting headache should raise suspicion,particularly in the presence of an underlying prothromboticcondition. Diagnosis is based on neuroimaging(MRI plus MRA or CT scan plus CTangiography or intra-arterial angiography in doubtfulcases). Treatment should be started as early aspossible and includes symptomatic treatment,heparin followed by at least 6 months of oral anticoagulationand, whenever indicated, treatment ofthe underlying cause.6.7 Headache attributed to other intracranial vasculardisorder6.7.1 Cerebral Autosomal Dominant Arteriopathywith Subcortical Infarcts and Leukoencephalopathy(CADASIL)Diagnosing headache type diagnostic criteria:A. Attacks of migraine with aura, with or withoutother neurological signsB. Typical white matter changes on MRI T2WIC. Diagnostic confirmation from skin biopsy evidenceor genetic testing (Notch 3 mutations)Diagnosing headache type comment:CADASIL is a recently identified autosomal dominant(with some sporadic cases) small artery diseaseof the brain characterised clinically by recurrentsmall deep infarcts, subcortical dementia, mood disturbancesand migraine with aura.Migraine with aura is present in one third of casesand, in such cases, is usually the first symptom ofthe disease, appearing at a mean age of 30, some 15years before ischaemic strokes and 20–30 yearsDiagnosing headache type (IHS 2003)72 ICHD-IIbefore death. Attacks are typical of 1.2 Migraine withaura except for an unusual frequency of prolongedaura.MRI is always abnormal with striking whitematter changes on T2WI. The disease involves thesmooth muscle cells in the media of small arteriesand it is due to mutations of Notch 3 gene. The diagnosisis made on a simple skin biopsy withimmunostaining of Notch 3 antibodies.CADASIL is an excellent model to study thepathophysiology of migraine with aura and the relationshipsbetween it and ischaemic stroke.6.7.2 Mitochondrial Encephalopathy, LacticAcidosis and Stroke-like episodes (MELAS)Diagnosing headache type diagnostic criteria:A. Attacks of migraine with or without auraB. Stroke-like episodes and seizuresC. Genetic abnormality (3243 point mitochondrialDNA mutation in the tRNA Leu gene or otherDNA MELAS point mutation)Diagnosing headache type comment:Migraine attacks are frequent in MELAS and this hasled to the hypothesis that mitochondrial mutationscould play a role in migraine with aura but the 3243mutation was not detected in two groups of subjectswith migraine with aura. Other yet-undetectedmutations may play a role in both migraine andischaemic stroke since migraine attacks, mostly withaura, also occur in other mitochondrial disorders.6.7.3 Headache attributed to benign (or reversible)angiopathy of the central nervous systemDiagnosing headache type diagnostic criteria:A. Diffuse, severe headache of abrupt or progressiveonset, with or without focal neurological deficitsand/or seizures and fulfilling criteria C and DB. ‘Strings and beads’ appearance on angiographyand subarachnoid haemorrhage ruled out byappropriate investigationsC. One or both of the following:1. headache develops simultaneously with neurologicaldeficits and/or seizures2. headache leads to angiography and discoveryof ‘strings and beads’ appearanceD. Headache (and neurological deficits, if present)resolves spontaneously within 2 monthsDiagnosing headache type comments:This is a poorly understood condition characterisedclinically by a severe diffuse headache of variablemodes of onset: it can be abrupt, mimicking SAH, orprogressive rapidly over hours or more slowly overdays. It is one of the identified causes of thunderclapheadache. It can be the only symptom of this conditionbut it is usually associated with fluctuatingfocal neurological deficits and sometimes seizures.Angiography is, by definition, abnormal, withalternating segments of arterial constriction anddilatation.Anumber of causes have been identified: the bestdefined is post-partum angiopathy which has beenrelated in some cases to use of bromocriptine. Thedisease is self-limiting in 1–2 months without treatmentand with disappearance of the arterial abnormalitiesbut, given the diagnostic difficulty withprimary CNS angiitis, a course of steroids is sometimesgiven.6.7.4 Headache attributed to pituitary apoplexyDiagnosing headache type diagnostic criteria:A. Severe acute retro-orbital, frontal or diffuseheadache accompanied by at least one of the followingand fulfilling criteria C and D:1. nausea and vomiting2. fever3. diminished level of consciousness4. hypopituitarism5. hypotension6. ophthalmoplegia or impaired visual acuityB. Neuroimaging evidence of acute haemorrhagicpituitary infarctionC. Headache develops simultaneously with acutehaemorrhagic pituitary infarctionD. Headache and other symptoms and/or signsresolve within 1 monthDiagnosing headache type comment:This rare clinical syndrome is an acute, lifethreateningcondition, characterised by spontaneoushaemorrhagic infarction of the pituitary gland. It isone of the causes of thunderclap headache.Magnetic resonance imaging is more sensitivethan CT scan for detecting intrasellar pathology.Bibliography6.1.1 Headache attributed to ischaemic strokeFerro JM, Melo TP, Oliveira V, Salgado AV, Crespo M, CanhaoP, Pinto AN. A multivariate study of headache associatedwith ischemic stroke. Headache 1995; 35:315–9.Fisher CM. Headache in acute cerebrovascular disease. In:Vinken PH, Bruyn GW eds. Headache and cranial neuralgias.Handbook of clinical neurology. Amsterdam; Elsevier1968; 5:124–56.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 73Gorelick PB, Hier DB, Caplan LR, Langenberg D. Headachein acute cerebrovascular disease. Neurology 1986; 36:1445–50.Portenoy RK, Abissi CJ, Lipton RB, Berger AR, Mebler MF,Baglivo J, Solomon S. Headache in cerebrovascular disease.Stroke 1984; 15:1009–12.6.1.2 Headache attributed to transient ischaemicattackCaplan LR. Migraine and vertebrobasilar ischemia. Neurology1991; 41:55–61.Ferro JM, Costa I, Melo TP, Canhao P, Oliveira V, Salgado AV,Crespo M, Pinto AN. Headache associated with transientischemic attacks. Headache 1995; 35:544–8.Fisher CM. Migraine accompaniments versus arterioscleroticischemia. Trans Am Neurol Assoc 1968; 93:211–3.Fisher CM. Cerebral ischemia: less familiar types. Clin Neurosurg1971; 18:267–336.Fisher CM. Late-life migraine accompaniements as a cause ofunexplained transient ischemic attacks. Can J Med Sci 1980;7:9–17.Martsen BH, Sorensen PS, Marquardsen J. Transient ischemicattacks in young patients: a thromboembolic or migrainousmanifestation? Aten-year follow-up of 46 patients. J NeurolNeurosurg Psychiat 1990; 53:1029–33.6.2.1 Headache attributed to intracerebralhaemorrhageFerro JM, Melo TP, Guerreiro M. Headaches in intracerebralhemorrhage survivors. Neurology 1998; 50:203–7.Gorelick PB, Hier DB, Caplan LR, Langenberg P. Headache inacute cerebrovascular disease. Neurology 1986; 36:1445–0.Jensen TS, Gorrelick PB. Headache associated with stroke andintracranial hematoma. In: The Headaches. 2nd edition.J Olesen, P Telt-Hansen, KMA Welch eds. Philadelphia:Lippincott Williams and Wilkins 2000, pp 781–7.Melo TP, Pinto AN, Ferro JM. Headache in intracerebralhematomas. Neurology 1996; 47:494–500.Schuaib A, Metz L, and Hing T. Migraine and intra-cerebralhemorrhage. Cephalalgia 1989; 9:59–61.6.2.2 Headache attributed to subarachnoidhaemorrhageBassi P, Bandera R Loiero M, Togoni G, Mangoni. Warningsigns in subarachnoid hemorrhage: a cooperative tudy. ActaNeurol Scand 1991; 84:277–81.Edlow JA, Caplan LR. Avoiding pitfalls in the diagnosis ofsubarachnoid hemorrhage. NEJM 2000; 342:29–36.Evans RW. Diagnostic testing for the evaluation of headaches.Neurology Clinics 1996; 14:1–26.Linn FHH, Rinkel GJE, Algra A, van Gijn J. Headache characteristicsin subarachnoid haemorrhage and benign thunderclapheadache. J Neurol Neurosurg Psych 1998; 65:791–3.Mayberg MR, Batjer HH, Dacey R, Diringer M, Haley EC,Heros RC, Sternau LL, Torner J, Adams HP, Feinberg W,Thies W. Guidelines for the management of aneurysmalsubarachnoid hemorrhage. A statement for healthcareprofessionals from a special writing group of the StrokeCouncil of the American Heart Association. Stroke 1994;25:2315–28.Ramirez-Lassepas M, Espinosa CE, Cicero JJ, Johnston KL,Cipolle RJ, Barber DL. Predictors of intracranial pathologicfindings in patients who seek emergency care because ofheadache. Arch Neurol 1997; 54:1506–9.Seymour JJ, Moscati RM, Jehle DV. Response of headaches tonon-narcotic analgesics resulting in missed intracranialhemorrhage. Am J Emerg Med 1995; 13:43–5.Sidman R, Vconnolly E, Lemke T. Subarachnoid hemorrhagediagnosis: lumbar puncture is still needed when the computedtomography scan is normal. Acad Emerg Med 1996;3:827–31.Van der Wee N, Rinkel GJE, Hasan D, van Gijn J. Detection ofsubarachnoid hemorrhage on early CT: is lumbar puncturestill needed after a negative scan? J Neurol Neurosurg Psychiat1995; 58:357–9.Verweij RD, Wijdicks EFM, van Gijn J. Warning headache inaneurysmal subarachnoid hemorrhage. A case controlstudy. Arch Neurol 1988; 45:1019–20.Weir B. Diagnostic aspects of SAH. In: Weir B. Subarachnoidhemorrhage: causes and cures. New York: Oxford UniversityPress 1998, pp 144–76.6.3.1 Headache attributed to saccular aneurysmDay JW, Raskin NH. Thunderclap headache: symptom ofunruptured cerebral aneurysm. Lancet 1986; 2:1247–8.Linn FHH, Wijdicks EFM, van der Graaf Y, Weerdesteyn-vanVliet FAC, Bartelds A, van Gijn J. Prospective study of sentinelheadache in aneurysmal subarachnoid haemorrhage.Lancet 1994; 344:590–3.Markus HS. Aprospective follow-up of thunderclap headachemimicking subarachnoid haemorrhage. J Neurol NeurosurgPsych 1991; 54:1117–25.Mas JL, Baron JC, Bousser MG, Chiras J. Stroke, migraineand inracranial aneurysm: a case report. Stroke 1986;17:1019–21.Ostergard JR, Ramadan N. Unruptured vascular malformationsand subarachnoid hemorrhage. In The Headaches 2ndedition. J Olesen, P Tfelt-Hansen, KMAWelch eds. Philadelphia:Lipincott Williams and Wilkins 2000: pp 789–96.Raps EC, Rogers JD, Galetta DL, Solomon RA, Lennihan L,Klebanoff LM, Fink ME. The clinical spectrum of unrupturedintracranial aneurysms. Arch Neurol 1993; 50:265–8.Schievink WI. Intracranial aneurysms. NEJM 1997; 336:28–40.Wijdicks EFM, Kerkhoff H, van Gijn J. Long-term follow-upof 71 patients with thunderclap headache mimicking subarachnoidhaemorrhage. Lancet 1988; ii:68–70.6.3.2 Headache attributed to arteriovenousmalformationBruyn GW. Intracranial arteriovenous malformation andmigraine. Cephalalgia 1984; 4:191–207.Haas DC. Arteriovenous malformations and migraine: casereports and an analysis of the relationship. Headache 1991;31:509–13.Troost BT, Mark LE, Maroon JC. Resolution of classic migraineafter removal of an occipital lobe AVM. Ann Neurol 1979;5:199–201.Diagnosing headache type (IHS 2003)74 ICHD-II6.3.3 Headache attributed to dural arterio-venousfistulaMalek AM, Halbach VV, Dowd CF, Higashida RT. Diagnosisand treatment of dural arteriovenous fistulas. NeuroimagingClin N Am 1998; 8:445–68.6.3.4 Headache attributed to cavernous angiomaDe Benedittis G. SUNCT syndrome associated with cavernousangioma of the brain stem. Cephalalgia 1996; 16:503–6.Epstein MA, Beerman PH, Schut L. Cavernous angioma presentingas atypical facial and head pain. J Child Neurol1990; 5:27–30.Robinson JR, Awad IA, Little JR. Natural history of thecavernous angioma. J Neurosurg 1991; 75:709–14.6.3.5 Headache attributed to encephalotrigeminalangiomatosis (Sturge Weber syndrome)Chabriat H, Pappata S, Traykov L, Kurtz A, Bousser MG.Angiomatose de Sturge Weber responsable d’une hémiplégiesans infarctus cérébral en fin de grossesse. RevNeurol (Paris) 1996; 152:536–41.Klapper J. Headache in Sturge-Weber syndrome. Headache1994; 34:521–2.6.4.1 Headache attributed to giant cell arteritisCaselli RJ, Hunder GG. Neurologic aspects of giant cell (temporal)arteritis. Rheum Dis Clin North Am 1993; 19:941–53.Hunder GG. Giant cell (temporal) arteritis. Rheum Dis ClinNorth Am 1990; 16:399–409.Lee AG, Brazis PW. Temporal arteritis: a clinical approach. JAm Geriatr Soc 1999; 47:1364–70.Solomon S, Cappa KG. The headache of temporal arteritis. JAm Geriatr Soc 1987; 35:163–5.Swannell AJ. Polymyalgia rheumatica and temporal arteritis:diagnosis and management. BMJ 1997; 314:1329–32.Thielen KR, Wydicks EFM, Nichols DA. Giant cell (temporal)arteritis: involvement of the vertebral and internal carotidarteries. Mayo Clin Proc 1998; 73:444–6.6.4.2 Headache attributed to Primary CNS angiitisand 6.4.3 Headache attributed to Secondary CNSangiitisCalabrese LH, Furlan AH, Gragg LA, Ropos TH. Primaryangiitis of the central nervous system: diagnostic criteriaand clinical approach. Cleve J Med 1992: 59:293–306.Calabrese LH, Duna GF, Lie JT. Vasculitis in the centralnervous system; Arthritis Rheum 1997; 40:1189–201.Chu CT, Gray L, Goldstein LB, Hulette CM. Diagnosis ofintracranial vasculitis: a multidisciplinary approach. J NeuropathExp Neurol 1998; 57:30–8.Hankey GJ. Necrotizing and granulomatous angiitis of theCNS. In Cerebrovascular disease. Pathophysiology, diagnosisand management, vol 2. MD Ginsberg, J Bogousslavskyeds. New York: Blackwell Science 1998: 1647–83.Harris KG, Tran DD, Sickels WJ, Cornell SH, Yuh WTC. Diagnosingintracranial vasculitis: the roles or MR and angiography.Am J Neuroradiol 1994; 15:317–30.Kumar R, Wijdicks EFM, Brown RD, Parisi JE, Hammond CA.Isolated angiitis of the CNS presenting as subarachnoidhaemorrhage. J Neurol Neurosurg Psych 1997; 62:649–51.Lie JT. Primary (granulomatous) angiitis of the central nervoussystem: a clinicopathologic analysis of 15 new cases and areview of the literature. Hum Pathol 1992; 23:164–71.Moore PM. Vasculitis of the central nervous system. SeminNeurol 1994; 14: 313–9.Savage COS, Harper L, Cockwell P, Adu D, Howie AJ. ABCof arterial and vascular disease: vasculitis. BMJ 2000;320:1325–8.6.5.1 Arterial dissectionBiousse V, D’Anglejan-Chatillon J, Massiou H, Bousser MG.Head pain in non traumatic artery dissection: a series of 65patients. Cephalalgia 1994; 14:33–6.Biousse V, D’Anglejan-Chatillon J, Touboul PJ, Amarenco P,Bousser MG. Time course of symptoms in extracranialcarotid artery dissections. A series of 80 patients. Stroke1995; 26:235–9.Biousse V, Woimant F, Amarenco P, Touboul PJ, Bousser MG.Pain as the only manifestation of extracranial internalcarotid artery dissection. Cephalalgia 1992; 12:314–7.D’Anglejan Chatillon J, Ribeiro V, Mas JL, Youl BD, BousserMG. Migraine – a risk factor for dissection of cervical arteries.Headache 1989; 29:560–1.De la Sayette V, Leproux F, Letellier Ph. Cervical cord anddorsal medullary infarction presenting with retro-orbitalpain. Neurology 1999; 53:632–4.Duyff RF, Snidjers CJ, Vanneste JAL. Spontaneous bilateralinternal carotid artery dissection and migraine: a potentialdiagnostic delay. Headache 1997; 37:109–12.Fisher CM. The headache and pain of spontaneous carotiddissection. Headache 1982; 22:60–5.Guillon B, Biousse V, Massiou H, Bousser MG. Orbital pain asan isolated sign of internal carotid artery dissection. Adiagnosticpitfall. Cephalagia 1998, 18:222–4.Guillon B, Lévy C, Bousser MG. Internal carotid artery dissection:an update. J Neurol Sci 1998; 153:146–58.Nakatomi H, Nagata K, Kawamoto S, Shiokawa Y. Ruptureddissecting aneurysm as a cause of subarachnoid hemorrhageof unverified etiology. Stroke 1997; 28:1278–82.Ramadan NM, Tietjen GE, Levine SR, Welch KMA. Scintillatingscotomata associated with internal carotid artery dissection:report of three cases. Neurology 1991; 41:1084–7.Silbert PL, Mokri B, Schievink WI. Headache and neck painin spontaneous internal carotid and verterbral artery dissections.Neurology 1995; 45:1517–22.Sturzenegger M. Headache and neck pain. The warningsymptoms of vertebral artery dissection. Headache 1994;34:187–93.6.5.2 Post-endarterectomy headacheBreen JC, Caplan LR, DeWitt LD, Belkin M, Mackey WC,Donnell TP. Brain edema after carotid surgery. Neurology1996; 46:175–81.De Marinis M, Zaccaria A, Faraglia V, Fiorani P, Maira G,Agnoli A. Post endarterectomy headache and the role of theoculo-sympathetic system. J Neurol Neurosurg Psych 1991;54:314–7.Ille O, Woimant F, Pruna A, Corabianu O, Idatte JM, HaguenauM. Hypertensive encephalopathy after bilateral carotidendarterectomy. Stroke 1995; 26:488–91.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 75Leviton A, Caplan L, Salzman E. Severe headache after carotidendarterectomy. Headache 1975; 15:207–9.Tehindrazanarivelo A, Lutz G, Petitjean C, Bousser MG.Headache following carotid endarterectomy: a prospectivestudy. Cephalalgia 1991; 11(suppl 11):353.6.5.3 Carotid angioplasty headacheDietrich EB, Ndiaye M, Reid DB. Stenting in the carotid artery.Experience in 110 patients. J Endovasc Surg 1996; 3:42–62.Gil-Peralta A, Mayol A, Gonzalez Marcos JR, Gonzalez A,Ruano J, Boza F, Duran F. Percutaneous transluminal angioplastyof the symptomatic atherosclerotic carotid arteries;Results, complications and follow-up. Stroke 1996; 27:2271–3.McCabe DJH, Brown MM, Clifton A. Fatal cerebral reperfusionhemorrhage after carotid stenting. Stroke 1999;30:2483–6.Munari LM, Belloni G, Moschini L, Mauro A, Pezzuoli G,Porta M. Carotid pain during percutaneous angioplasty.Pathophysiology and clinical features. Cephalalgia 1994;14:127–31.Schoser BG, Heesen C, Eckert B, Thie A. Cerebral hyperperfusioninjury after percutaneous transluminal angioplastyof extracranial arteries. J Neurol 1997; 244:101–4.6.5.4 Headache attributed to intracranialendovascular proceduresMartins IP, Baeta E, Paiva T, Campos J, Gomes L. Headachesduring intracranial endovascular procedures: a possiblemodel for vascular headache. Headache 1993; 23:227–33.Nichols FT, Mawad M, Mohr JP, Hilal S, Adams RJ. Focalheadache during balloon inflation in the vertebral andbasilar arteries. Headache 1993; 33:87–9.Nichols FT, Mawad M, Mohr JP, Stein B, Hilal S, MichelsenWJ. Focal headache during balloon inflation in the internalcarotid and middle cerebral arteries. Stroke 1990; 21:555–9.6.5.5 Angiography headacheShuaib A, Hachinski VC. Migraine and the risks from angiogaphy.Arch Neurol 1988; 45:911–2.6.6 Headache attributed to cerebral venousthrombosisAidi S, Chaunu MP, Biousse V, Bousser MG. Changing patternof headache pointing to cerebral venous thrombosis afterlumbar puncture and intra venous high dose corticosteroids.Headache 1999; 39:559–64.Ameri A, Bousser MG. Cerebral venous thrombosis. NeurologicClinics 1992; 10:87–111.Biousse V, Ameri A, Bousser MG. Isolated intracranial hypertensionas the only sign of cerebral venous thrombosis.Neurology 1999; 53:1537–42.Bousser MG, Ross Russell R. Cerebral venous thrombosis.Major problems in neurology, vol 1. London: Saunders 1997.Crassard I, Biousse V, Bousser MG, Meyer B, Marsot-DupuchK. Hearing loss and headache revealing lateral sinus thrombosisin a patient with Factor V Leiden mutation. Stroke1997; 28:876–7.De Bruijn SFTM, Stam J, Kappelle LJ for CVST study group.Thunderclap headache as first symptom of cerebral venoussinus thrombosis. Lancet 1996; 348:1623–5.Leker RR, Steiner I. Features of dural sinus thrombosis simulatingpseudotumor cerebri. Eur J Neurol 1999; 6:601–4.Newman DS, Levine SR, Curtis VL, Welch KMA: Migrainelike visual phenomena associated with cerebral venousthrombosis. Headache 1989; 29:82–5.Tehindrazanarivelo AD, Evrard S, Schaison M, Mas JL,Dormont D, Bousser MG: Prospective study of cereral sinusvenous thrombosis in patients presenting with benignintracranial hypertension. Cerebrovasc Dis 1992; 2:22–7.6.7.1 CADASILChabriat H, Tournier-Lasserve E, Vahedi K, Leys D, Joutel A,Nibbio A, Escailla JP, Iba-Zizen MT, Bracard S, TehindrazanariveloA, Gastaut JL, Bousser MG. Autosomal dominantmigraine with MRI white matter abnormalities mapping tothe CADASIL locus. Neurology 1995; 45:1086–91.Chabriat H, Vahedi K, Iba-Zizen MT, Joutel A, Nibbio A, NagyTG, Krebs MO, Julien J, Dubois B, Ducrocq X, Levasseur M,Homeyer P, Mas JL, Lyon-Caen O, Tournier-Lasserve E,Bousser MG. Clinical spectrum of CADASIL: a study of 7families. Lancet 1995; 346:934–9.Joutel A, Corpechot C, Ducros A, Katayoun V, Chabriat H,Mouton P, Alamowitch S, Domenga V, Cécillion M,Maréchal E, Maciazek J, Vayssière C, Cruaud C, CabanisEA, Ruchoux MM, Weissenbach J, Bacxh JF, Bousser MG,Tournier-Lasserve E. ‘Notch 3’ mutations in CADASIL, ahereditary adult-onset condition causing stroke anddementia. Nature 1996; 383:707–10.Verin M, Rolland Y, Landgraf F Chabriat H, Bompais B, MichelA, Vahedi K, Martinet JP, Tournier-Lasserve E, LemaitreMH, Edan G. New phenotype of the cerebral autosomaldominant arteriopathy mapped to chromosome 19migraine as the prominent clinical feature. J Neurol NeurosurgPsychiat 1995; 59:579–85.6.7.2 MELASKlopstock A, May P, Siebel E, Papagiannuli E, Diener NC,Heichmann H. Mitochondrial DNA in migraine with aura.Neurology 1996; 46:1735–8.Koo B, Becker L, Chuang S, Merante F, Robinson BH, MacGregor D, Tein I, Ho VB, McGreal DA, Wherrett JR, LoganWJ. Mitochondrial encephalomyopathy, lactic acidosis,stroke-like episodes (MELAS): clinical, radiological, pathologicaland genetic observations. Ann Neurol 1993;34:25–32.Ojaimi J, Katsabanis S, Bower S, Quigley A, Byrne E. MitochondrialDNA in stroke and migraine with aura. CerebrovascDis 1998; 8:102–6.Pavlakis SG, Phillips PC, Di Mauro S, De Vivo DC, RowlandP. Mitochondrial myopathy, encephalopathy, lactic acidosisand stroke-like episodes: a distinct clinical syndrome. AnnNeurol 1984; 16:481–8.6.7.3 Headache attributed to benign angiopathy ofthe CNSCall GK, Fleming MC, Sealfon S, Levine H, Kistler JP, FisherCM. Reversible cerebral segmental vasoconstriction. Stroke1988; 19:1159–70.Diagnosing headache type (IHS 2003)76 ICHD-IIDodick DW, Brown RD, Britton JW, Huston J. Non aneurysmalthunderclap headache with diffuse, multifocal segmentaland reversible vasospasm. Cephalalgia 1999;19:118–213.Lee KY, Sohn YH, Kim SH, Sunwoo IN. Basilar arteryvasospasm in postpartum cerebral angiopathy. Neurology2000; 54:2003–5.McColl GJ, Fraser K. Pheochromocytoma and pseudovasculitis.J Rheumatol 1995; 22:1441–2.Razavi M, Bendixen B, Maley JE, Schoaib M, Zargarian M,Razavi B, Adams HP. CNS pseudovasculitis in a patientwith pheochromocytoma. Neurology 1999; 52:1088–90.Serdaru M, Chiras J, Cujas M, Lhermitte F. Isolated benigncerebral vasculitis or migrainous vasospasm? J Neurol NeurosurgPsychiat 1984; 47:73–6.6.7.4 Headache attributed to pituitary apoplexyCarral F. Pituitary apoplexy. Arch Neurol 2001; 58:1143–4.Chakeres DW, Curtin A, Ford G. Magnetic resonance imagingof pituitary and parasellar abnormalities. Radiol Clin NorthAm 1989; 27:265–81.Da Motta LA, de Mello PA, de Lacerda CM, Neto AP, DaMotta LD, Filho MF. Pituitary apoplexy. Clinical course,endocrine evaluations and treatment analysis. J NeurosurgSci 1991; 43:25–36.Dodick DW, Wijdicks EFM. Pituitary apoplexy presenting asthunderclap headache. Neurology 1998; 50:1510–1.Hernandez A, Angeles Del Real M, Aguirre M, Vaamonde J,Gudin M, Ibanez R. Pituitary apoplexy: a transient benignpresentation mimicking with subarachnoid hemorrhagewith negative angiography. Eur J Neurol 1998; 5:499–501.Lee CC, Cho AS, Carter WA. Emergency department presentationof pituitary apoplexy. Am J Emerg Med 2000; 18:328–31.McFadzean RM, Doyle D, Rampling R, Teasdale E, TeasdaleG. Pituitary apoplexy and its effect on vision. Neurosurgery1991; 29:669–75.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 777. Headache attributed to non-vascularintracranial disorder7.1 Headache attributed to high cerebrospinal fluidpressure7.1.1 Headache attributed to idiopathicintracranial hypertension (IIH)7.1.2 Headache attributed to intracranialhypertension secondary to metabolic,toxic or hormonal causes7.1.3 Headache attributed to intracranialhypertension secondary to hydrocephalus7.2 Headache attributed to low cerebrospinal fluidpressure7.2.1 Post-dural puncture headache7.2.2 CSF fistula headache7.2.3 Headache attributed to spontaneous (oridiopathic) low CSF pressure7.3 Headache attributed to non-infectiousinflammatory disease7.3.1 Headache attributed to neurosarcoidosis7.3.2 Headache attributed to aseptic (noninfectious)meningitis7.3.3 Headache attributed to other noninfectiousinflammatory disease7.3.4 Headache attributed to lymphocytichypophysitis7.4 Headache attributed to intracranial neoplasm7.4.1 Headache attributed to increasedintracranial pressure or hydrocephaluscaused by neoplasm7.4.2 Headache attributed directly to neoplasm7.4.3 Headache attributed to carcinomatousmeningitis7.4.4 Headache attributed to hypothalamic orpituitary hyper- or hyposecretion7.5 Headache attributed to intrathecal injection7.6 Headache attributed to epileptic seizure7.6.1 Hemicrania epileptica7.6.2 Post-seizure headache7.7 Headache attributed to Chiari malformationtype I (CM1)7.8 Syndrome of transient Headache andNeurological Deficits with cerebrospinal fluidLymphocytosis (HaNDL)7.9 Headache attributed to other non-vascularintracranial disorderGeneral commentPrimary or secondary headache or both?When a new headache occurs for the first time inclose temporal relation to a non-vascular intracranialdisorder, it is coded as a secondary headache attributedto the intracranial disorder. This is also true ifthe headache has the characteristics of migraine,tension-type headache or cluster headache. Whena pre-existing primary headache is made worse inclose temporal relation to an intracranial disorder,there are two possibilities, and judgment is required.The patient can either be given only the diagnosis ofthe pre-existing primary headache or be given boththis diagnosis and the diagnosis of headache attributedto the intracranial disorder. Factors that supportadding the latter diagnosis are: a very close temporalrelation to the intracranial disorder, a markedworsening of the pre-existing headache, very goodevidence that the intracranial disorder can aggravatethe primary headache and, finally, improvementor resolution of the headache after relief from theintracranial disorder.Definite, probable or chronic?A diagnosis of Headache attributed to non-vascularintracranial disorder usually becomes definite onlywhen the headache resolves or greatly improvesafter effective treatment or spontaneous remissionof the causative disorder. If the intracranial disordercannot be treated effectively or does not remit spontaneously,or when there has been insufficient timefor this to happen, a diagnosis of Headache probablyattributed to non-vascular intracranial disorder isusually applied.The alternative, when the causative disorder iseffectively treated or remits spontaneously butheadache does not resolve or markedly improveafter 3 months, is a diagnosis of A 7.10 Chronic postintracranialdisorder headache. This is described onlyin the appendix as such headaches have been poorlydocumented, and research is needed to establishbetter criteria for causation.IntroductionIn this chapter are the headaches attributed tochanges in intracranial pressure. Both increased anddecreased CSF pressure can lead to headache. Othercauses of headache here are non-infectious inflammatorydiseases, intracranial neoplasia, seizures,rare conditions such as intrathecal injections andChiari malformation type I, and other non-vascularintracranial disorders.Compared to those on primary headaches, thereare few epidemiological studies on these headachetypes. Controlled trials of therapy are almost nonexistent.Diagnosing headache type (IHS 2003)78 ICHD-IIHeadache persisting for more than 1 month aftersuccessful treatment or spontaneous resolution ofthe intracranial disorder usually has other mechanisms.Chronic headache persisting for >3 monthsafter treatment or remission of intracranial disordersis defined in the appendix for research purposes.Such headaches exist but have been poorly studiedand the appendix entries are intended to stimulatefurther research into such headaches and theirmechanisms.7.1 Headache attributed to high cerebrospinal fluidpressureCoded elsewhere:7.4.1 Headache attributed to increased intracranial pressureor hydrocephalus caused by neoplasm.7.1.1 Headache attributed to idiopathic intracranialhypertension (IIH)Diagnosing headache type previously used terms:Benign intracranial hypertension (BIH), pseudotumorcerebri, meningeal hydrops, serous meningitisDiagnosing headache type diagnostic criteria:A. Progressive headache with at least one of the followingcharacteristics and fulfilling criteria C andD:1. daily occurrence2. diffuse and/or constant (non-pulsating) pain3. aggravated by coughing or strainingB. Intracranial hypertension fulfilling the followingcriteria:1. alert patient with neurological examinationthat either is normal or demonstrates any ofthe following abnormalities:a) papilloedemab) enlarged blind spotc) visual field defect (progressive ifuntreated)d) sixth nerve palsy2. increased CSF pressure (>200mm H2O in thenon-obese, >250mm H2O in the obese) measuredby lumbar puncture in the recumbentposition or by epidural or intraventricularpressure monitoring3. normal CSF chemistry (low CSF protein isacceptable) and cellularity4. intracranial diseases (including venoussinus thrombosis) ruled out by appropriateinvestigations5. no metabolic, toxic or hormonal cause ofintracranial hypertensionC. Headache develops in close temporal relation toincreased intracranial pressureD. Headache improves after withdrawal of CSF toreduce pressure to 120–170mm H2O and resolveswithin 72 hours of persistent normalisation ofintracranial pressureDiagnosing headache type comments:IIH most commonly occurs in young obese women.Although the majority of patients with IIH havepapilloedema, IIH without papilloedema isobserved. Other symptoms or signs of IIH includeintracranial noises, tinnitus, transient visual obscurationsand diplopia.7.1.2 Headache attributed to intracranialhypertension secondary to metabolic, toxic orhormonal causesCoded elsewhere:Headache attributed to increased intracranial pressuredue to head trauma, vascular disorder orintracranial infection is coded to whichever one ofthose disorders is present. Headache attributed toraised intracranial pressure occurring as a side-effectof medication is coded as 8.3 Headache as an adverseevent attributed to chronic medication.Diagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. daily occurrence2. diffuse and/or constant (non-pulsating) pain3. aggravated by coughing or strainingB. Intracranial hypertension fulfilling the followingcriteria:1. alert patient with neurological examinationthat either is normal or demonstrates any ofthe following abnormalities:a) papilloedemab) enlarged blind spotc) visual field defect (progressive ifuntreated)d) sixth nerve palsy2. increased CSF pressure (>200mm H2O in thenon-obese, >250mm H2O in the obese) measuredby lumbar puncture in the recumbentposition or by epidural or intraventricularpressure monitoring3. normal CSF chemistry (low CSF protein isacceptable) and cellularity4. intracranial diseases (including venoussinus thrombosis) ruled out by appropriateinvestigationsDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 79C. Headache develops after weeks or months ofendocrine disorder, hypervitaminosis Aor intakeof substances (other than medications) that canelevate CSF pressureD. Headache resolves within 3 months after removalof the causeDiagnosing headache type comment:Normal pressure hydrocephalus does not causeheadache.7.1.3 Headache attributed to intracranialhypertension secondary to hydrocephalusDiagnosing headache type diagnostic criteria:A. Headache with at least two of the following characteristicsand fulfilling criteria C and D:1. diffuse pain2. worse in the morning3. worse with Valsalva-like manoeuvres4. accompanied by vomiting5. associated with papilloedema, sixth nervepalsy, altered level of consciousness, gait instabilityand/or increased head circumference (inchildren <5 years old)B. High-pressure hydrocephalus fulfilling the followingcriteria:1. ventricular enlargement on neuroimaging2. intracranial pressure >200mm H2O in the nonobeseor >250mm H2O in the obese3. no other intracranial disorder causingincreased CSF pressureC. Headache develops in close temporal relation toincreased CSF pressureD. Headache resolves within 72 hours of normalisationof CSF pressure7.2 Headache attributed to low cerebrospinal fluidpressure7.2.1 Post-dural (post-lumbar) puncture headacheDiagnosing headache type diagnostic criteria:A. Headache that worsens within 15 minutes aftersitting or standing and improves within 15minutes after lying, with at least one of the followingand fulfilling criteria C and D:1. neck stiffness2. tinnitus3. hypacusia4. photophobia5. nauseaB. Dural puncture has been performedC. Headache develops within 5 days after duralpunctureD. Headache resolves either1:1. spontaneously within 1 week2. within 48 hours after effective treatment of thespinal fluid leak (usually by epidural bloodpatch)Note:1. In 95% of cases this is so. When headache persists,causation is in doubt.7.2.2 CSF fistula headacheDiagnosing headache type diagnostic criteria:A. Headache that worsens within 15 minutes aftersitting or standing, with at least one of thefollowing and fulfilling criteria C and D:1. neck stiffness2. tinnitus3. hypacusia4. photophobia5. nauseaB. A known procedure or trauma has caused persistentCSF leakage with at least one of thefollowing:1. evidence of low CSF pressure on MRI (eg,pachymeningeal enhancement)2. evidence of CSF leakage on conventionalmyelography, CT myelography or cisternography3. CSF opening pressure <60mm H2O in sittingpositionC. Headache develops in close temporal relation toCSF leakageD. Headache resolves within 7 days of sealing theCSF leak7.2.3 Headache attributed to spontaneous (oridiopathic) low CSF pressureDiagnosing headache type previously used terms:Spontaneous intracranial hypotension, primaryintracranial hypotension, low CSF-volume headache,hypoliquorrhoeic headacheDiagnosing headache type diagnostic criteria:A. Diffuse and/or dull headache that worsenswithin 15 minutes after sitting or standing, withat least one of the following and fulfilling criterionD:1. neck stiffness2. tinnitus3. hypacusia4. photophobia5. nauseaDiagnosing headache type (IHS 2003)80 ICHD-IIB. At least one of the following:1. evidence of low CSF pressure on MRI (eg,pachymeningeal enhancement)2. evidence of CSF leakage on conventionalmyelography, CT myelography or cisternography3. CSF opening pressure <60mm H2O in sittingpositionC. No history of dural puncture or other cause ofCSF fistulaD. Headache resolves within 72 hours after epiduralblood patchingDiagnosing headache type comments:The underlying disorder may be low CSF volume. Ahistory of trivial increase in intracranial pressure (eg,on vigorous coughing) is often elicited. In other casesa sudden drop in atmospheric pressure hasoccurred.Postural headache resembling that of low CSFpressure has been reported after coitus. Suchheadache should be coded here because it is due toCSF leakage.Many patients with spontaneous low CSF pressureheadache respond to epidural blood patching,epidural saline infusion or pharmacological therapiessuch as intravenous caffeine or conventionalanalgesics. Some have spontaneous resolution oftheir headache, while others relapse after initial successfultreatment. Cases of dural sleeve herniation,particularly in the thoracic area, have been reportedand have been successfully treated surgically.Dural puncture should be avoided in patients withpositive MRI signs such as meningeal enhancementwith contrast.7.3 Headache attributed to non-infectious inflammatorydisease7.3.1 Headache attributed to neurosarcoidosisDiagnosing headache type diagnostic criteria:A. Headache, no typical characteristics known, fulfillingcriteria C and DB. Evidence of neurosarcoidosis1C. Headache develops in temporal relation toneurosarcoidosisD. Headache resolves within 3 months after successfultreatment of neurosarcoidosisNote:1. Evidence of neurosarcoidosis includes cranialnerve lesions, intracranial space-occupying lesionon MRI, aseptic meningitis and/or periventricularinflammatory focal lesions and homogeneouslyenhancing mass lesions that areconfirmed on biopsy as non-caseating granulomas.7.3.2 Headache attributed to aseptic (noninfectious)meningitisDiagnosing headache type diagnostic criteria:A. Diffuse headache fulfilling criterion DB. Examination of CSF shows lymphocytic pleocytosis,mildly elevated protein and normal glucosein the absence of infectious organismsC. Use of one of the following: ibuprofen,immunoglobulins, penicillin or trimethoprim,intrathecal injections or insufflationsD. Headache resolves within 3 months after withdrawalof the offending substance7.3.3 Headache attributed to other non-infectiousinflammatory diseaseDiagnosing headache type diagnostic criteria:A. Headache, no typical characteristics known, fulfillingcriteria C and DB. Evidence of one of the inflammatory diseasesknown to be associated with headache1C. Headache develops in close temporal relation tothe inflammatory disorderD. Headache resolves within 3 months after successfultreatment of the inflammatory disorderNote:1. Headache can be associated with but is notusually a presenting or dominant symptom ofacute demyelinating encephalomyelitis (ADEM),systemic lupus erythematosus (SLE), Behçet’ssyndrome, anti-phospholipid antibody syndrome,Vogt-Koyanagi-Harada syndrome.7.3.4 Headache attributed to lymphocytichypophysitisDiagnosing headache type diagnostic criteria:A. Headache, no typical characteristics known, fulfillingcriterion CB. Hypopituitarism fulfilling the following criteria:1. MRI demonstrates symmetrical pituitaryenlargement with homogeneous contrastenhancement2. biopsy confirmation of lymphocytichypophysitisDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 81C. Headache develops in close temporal relation tohypopituitarismDiagnosing headache type comments:Lymphocytic hypophysitis is often accompanied byhyperprolactinaemia (50% of cases) or autoantibodiesagainst hypophyseal cytosol protein (20%).This disorder typically develops at the end ofpregnancy or during the post-partum period, but itcan occur in men.7.4 Headache attributed to intracranial neoplasm7.4.1 Headache attributed to increased intracranialpressure or hydrocephalus caused by neoplasmDiagnosing headache type diagnostic criteria:A. Diffuse non-pulsating headache with at least oneof the following characteristics and fulfilling criteriaC and D:1. associated with nausea and/or vomiting2. worsened by physical activity and/ormanoeuvres known to increase intracranialpressure (such as Valsalva manoeuvre, coughingor sneezing)3. occurring in attack-like episodes1B. Space-occupying intracranial tumour demonstratedby CT or MRI and causing hydrocephalus2C. Headache develops and/or deteriorates in closetemporal relation to the hydrocephalusD. Headache improves within 7 days after surgicalremoval or volume-reduction of tumourNotes:1. Onset of headache can be sudden (thunderclapheadache) and, in such cases, associated with lossof consciousness.2. For example, colloid cyst of the IIIrd ventricle.7.4.2 Headache attributed directly to neoplasmDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. progressive2. localised3. worse in the morning4. aggravated by coughing or bending forwardB. Intracranial neoplasm shown by imagingC. Headache develops in temporal (and usuallyspatial) relation to the neoplasmD. Headache resolves within 7 days after surgicalremoval or volume-reduction of neoplasm ortreatment with corticosteroids7.4.3 Headache attributed to carcinomatousmeningitisDiagnosing headache type diagnostic criteria:A. Diffuse or localised headache fulfilling criterionC

B. Carcinomatous meningitis proven by (repeated)CSF examination and/or dural enhancement onMRIC. Headache develops and/or deteriorates withadvancing diseaseDiagnosing headache type comment:Headache may improve temporarily with intrathecalchemotherapy or prednisone (prednisolone).7.4.4 Headache attributed to hypothalamic orpituitary hyper- or hyposecretionDiagnosing headache type diagnostic criteria:A. Bilateral, frontotemporal and/or retro-orbitalheadache fulfilling criteria C and DB. At least one of the following:1. prolactin, growth hormone (GH) and adrenocorticotropichormone (ACTH) hypersecretionassociated with microadenomas <10mm indiameter2. disorder of temperature regulation, abnormalemotional state, altered thirst and appetite andchange in level of consciousness associatedwith hypothalamic tumourC. Headache develops during endocrineabnormalityD. Headache resolves within 3 months after surgicalresection or specific and effective medicaltherapy7.5 Headache attributed to intrathecal injectionDiagnosing headache type diagnostic criteria:A. Diffuse headache remaining present in therecumbent position and fulfilling criteria C andD

B. Intrathecal injection has been givenC. Headache develops within 4 hours after intrathecalinjectionD. Headache resolves within 14 days1Note:1. If headache persists beyond 14 days, the likelydiagnosis is 7.2.2 CSF fistula headache.Diagnosing headache type (IHS 2003)82 ICHD-II7.6 Headache attributed to epileptic seizureDiagnosing headache type comment:The association between migraine and epilepsy iscomplex and bi-directional. It may be related togenetic and/or environmental risk factors thatincrease neuronal excitability or decrease the thresholdto both types of attacks. Migraine and epilepsymay coexist without either being a contributing riskfactor for the other. Migraine and epilepsy may beco-morbid as certain brain disorders (eg, MELAS)predispose patients to both epilepsy and migraineoccurring remotely from each other. There appearsalso to be a high incidence of migraine in certainforms of epilepsy such as benign occipital epilepsy,benign rolandic epilepsy and corticoreticularepilepsy with absence seizures. Furthermore, structurallesions such as arteriovenous malformationsmay present with clinical features of migrainewith aura along with seizures, usually accompaniedby headache. Finally, seizures have been reportedto occur during or immediately following a migraineaura. The term migralepsy has been used to denoteepileptic seizures occurring between the migrainousaura and the headache phase of migraine. Thereshould be no reason why epileptic seizures, so vulnerableto extrinsic and intrinsic precipitatingfactors, could not be susceptible to cortical changesinduced by migraine. However, this is so extremelyrare that only a few case reports have been publisheddespite that migraine and epilepsy are among thecommonest brain diseases. According to a recentreview, most of these are genuine occipital seizuresimitating migraine aura. For example, two of thethree ‘migralepsy’ patients of Lennox and Lennox(1960) seemed to have symptomatic and idiopathicoccipital epilepsy with visual hallucinations.7.6.1 Hemicrania epilepticaDiagnosing headache type diagnostic criteria:A. Headache lasting seconds to minutes, with featuresof migraine, fulfilling criteria C and DB. The patient is having a partial epileptic seizureC. Headache develops synchronously with theseizure and is ipsilateral to the ictal dischargeD. Headache resolves immediately after the seizureDiagnosing headache type comment:Synchronous ipsilateral headache with migrainousfeatures occurring as an ictal manifestation of theseizure discharge is recognised, albeit rare. Diagnosisrequires the simultaneous onset of headache withelectroencephalographically-demonstrated ictaldischarge.7.6.2 Post-ictal headacheDiagnosing headache type diagnostic criteria:A. Headache with features of tension-type headacheor, in a patient with migraine, of migraineheadache and fulfilling criteria C and DB. The patient has had a partial or generalisedepileptic seizureC. Headache develops within 3 hours following theseizureD. Headache resolves within 72 hours after theseizureDiagnosing headache type comments:Post-ictal headache with migrainous features is awell-recognised consequence of a seizure discharge.Post-ictal headache is often indistinguishable frommigraine headache and associated with nausea andvomiting. It is equally common in those with orwithout a family history of migraine. Other similaritieswith migraine headache are that, in somepatients, post-ictal headache develops 3–15 minutesafter the end of visual hallucinations (and it is longerand more severe after visual seizures of longer duration).Similar post-ictal headache has been reportedin patients with symptomatic epilepsy but it ismainly emphasised in idiopathic occipital seizures.It may be that the seizure discharges in the occipitallobes trigger a genuine migraine headache throughtrigeminovascular or brainstem mechanisms.In a study of 100 patients with epilepsy, post-ictalheadache occurred in 51 and most commonly lasted6–72 hours. Major seizures were more often associatedwith post-ictal headache than were minorattacks. Nine patients in this series also hadmigraine: in eight, a typical albeit mild migraineattack was provoked by seizures. Post-ictal headachein the 43 who did not develop migraine was accompaniedby vomiting in 11 cases, photophobia in 14cases and vomiting with photophobia in 4 cases. Furthermore,post-ictal headache was accentuated bycoughing, bending and sudden head movementsand relieved by sleep. It is, therefore, clear thatseizures provoke a syndrome similar to theheadache phase of migraine in 50% of epileptics.7.7 Headache attributed to Chiari malformation type I(CM1)Diagnosing headache type diagnostic criteria:A. Headache characterised by at least one of the followingand fulfilling criterion D:Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 831. precipitated by cough and/or Valsalvamanoeuvre2. protracted (hours to days) occipital and/orsub-occipital headache3. associated with symptoms and/or signs ofbrainstem, cerebellar and/or cervical corddysfunctionB. Cerebellar tonsillar herniation as defined by oneof the following on craniocervical MRI:1. ≥5 mm caudal descent of the cerebellar tonsils2. ≥3 mm caudal descent of the cerebellar tonsilsplus at least one of the following indicators ofcrowding of the subarachnoid space in thearea of the craniocervical junction:a) compression of the CSF spaces posteriorand lateral to the cerebellumb) reduced height of the supraocciputc) increased slope of the tentoriumd) kinking of the medulla oblongataC. Evidence of posterior fossa dysfunction, based onat least two of the following:1. otoneurological symptoms and/or signs (eg,dizziness, disequilibrium, sensations ofalteration in ear pressure, hypacusia orhyperacusia, vertigo, down-beat nystagmus,oscillopsia)2. transient visual symptoms (spark photopsias,visual blurring, diplopia or transient visualfield deficits)3. demonstration of clinical signs relevant to cervicalcord, brainstem or lower cranial nervesor of ataxia or dysmetriaD. Headache resolves within 3 months after successfultreatment of the Chiari malformationDiagnosing headache type comments:Headache is often descriptively similar to primarycough headache with the exception of possiblylonger duration (minutes rather than seconds).Headache is the most common symptom of Chiarimalformation type I (CM1), but patients may alsohave localised vestibulo-ocular (74% of cases), lowercranial nerve, brainstem, cerebellar (50%) and/orspinal cord dysfunction suggestive of syringomyelia(66%). Although no specific criteria currently exist tocharacterise headache attributed to CM1, rigidadherence to the clinical and radiological criteriadescribed above is recommended prior to surgicalintervention. However, these criteria require validationand will inevitably be altered in future revisionsof The International Classification of Headache Disorders.Prospective studies with long-term surgicaloutcome are needed.7.8 Syndrome of transient Headache and NeurologicalDeficits with cerebrospinal fluid Lymphocytosis(HaNDL)Diagnosing headache type previously used terms:Migraine with cerebrospinal pleocytosis; pseudomigrainewith lymphocytic pleocytosisDiagnosing headache type diagnostic criteria:A. Episodes of moderate or severe headache lastinghours before resolving fully and fulfilling criteriaC and DB. Cerebrospinal fluid pleocytosis with lymphocyticpredominance (>15 cells/ml) and normal neuroimaging,CSF culture and other tests foraetiologyC. Episodes of headache are accompanied by orshortly follow transient neurological deficits andcommence in close temporal relation to the developmentof CSF pleocytosisD. Episodes of headache and neurological deficitsrecur over <3 monthsDiagnosing headache type comments:This syndrome, first clearly delineated by Bartlesonet al. (1981), has also been referred to in the literatureas a migrainous syndrome with cerebrospinalpleocytosis and as pseudomigraine with temporaryneurological symptoms and lymphocytic pleocytosis.The clinical picture is of one to >20 discreteepisodes of neurological deficits accompanied or followedby moderate to severe headache. Most of theepisodes last hours. The neurological manifestations,involving either cerebral hemisphere and/orthe brainstem/cerebellum, are most commonlysensory symptoms (78% of reported cases), aphasia(66%) and motor deficits (56%). Migraine-aura-likevisual symptoms are relatively uncommon (18%).Some individuals report a ‘march’ of symptomssimilar to that reported in typical migraine aura.Patients are asymptomatic between episodes.In addition to CSF lymphocytosis (10–760cells/ml), there are elevations of CSF total protein(20–250mg/dl) in >90% of cases and of the CSFopening pressure (100–400mm H20) in >50% ofcases. Papilloedema is occasionally present. RoutineCT and MRI scans (with or without intravenous contrast)and angiography are virtually always normal.Microbiological studies have been uniformlynormal. EEG and SPECT scans may show focallyabnormal areas consistent with the focal neurologicaldeficits.The CSF pleocytosis eventually normalises onrepeat sampling. Although no large systematic long-Diagnosing headache type (IHS 2003)84 ICHD-IIterm follow-up studies have been reported, itappears that some patients with this syndrome mayexperience recurrence of it.Most patients with this syndrome have no priorhistory of migraine. The clinician must considerother diagnoses that may share some of its clinicalfeatures, including familial hemiplegic migraine,neuroborreliosis, neurosyphilis, neurobrucellosis,mycoplasma, meningitis, granulomatous and neoplasticarachnoiditis, encephalitis and CNSvasculitis.7.9 Headache attributed to other non-vascularintracranial disorderDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. daily occurrence2. diffuse pain3. aggravated by Valsalva manoeuvreB. Evidence of an intracranial disorder other thanthose described aboveC. Headache develops in close temporal relation tothe intracranial disorderD. Headache resolves within 3 months after cureor spontaneous remission of the intracranialdisorderBibliography and references7.1 Headache attributed to high cerebrospinal fluidpressureCorbett JJ, Mehta MP. Cerebrospinal fluid pressure in normalobese subjects and patients with pseudotumor cerebri. Neurology1983; 33:1386–88.Corbett JJ, Nerad JA, Tse DT, Anderson RL. Results of opticnerve sheath fenestration for pseudotumor cerebri. Thelateral orbitotomy approach. Arch Ophthalmol 1988;106:1391–7.Corbett JJ, Thompson HS. The rational management of idiopathicintracranial hypertension. Arch Neurol 1989;46:1049–51.Digre KB, Corbett JJ. Pseudotumor cerebri in men. ArchNeurol 1988; 45:866–72.Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumorcerebri: population studies in Iowa and Louisiana. ArchNeurol 1988; 45:875–7.Eggenberger ER, Miller NR, Vitale S. Lumboperitoneal shuntfor the treatment of pseudotumor cerebri. Neurology 1996;46:1524–30.Fishman RA. Cerebrospinal fluid in diseases of the nervoussystem, WB Saunders Company, Philadelphia. 1992.Gamache FW, Patterson RH, Alksne JF. Headache associatedwith changes in intracranial pressure. In Wolff’s headacheand other head pain, (Dalessio DJ, ed.) Oxford UniversityPress, New York. 1987; pp. 352–5.Gardner K, Cox T, Digre K. Idiopathic intracranial hypertensionassociated with tetracycline use in fraternal twins: casereport and review. Neurology 1995; 45:6–10.Giuseffi V, Wall M, Siegal PZ, Rojas PB. Symptoms and diseaseassociations in idiopathic intracranial hypertension(pseudotumor cerebri): A case control study. Neurology1991; 41:239–44.Griffin JP. A review of the literature on benign intracranialhypertension. Adverse Drug React Toxicol Rev 1992;11:41–58.Johnson LN, Krohel GB, Madsen RW, March GA. The role ofweight loss and acetazolamide in the treatment of idiopathicintracranial hypertension (pseudotumor cerebri).Ophthalmol 1998; 105:2313–7.Johnston I, Paterson A. Benign intracranial hypertension II.CSF pressure and circulation. Brain 1974; 97:301–12.Karahalios DG, Rekate HL, Khayata MH, Apostolides PJ. Elevatedintracranial venous pressure as a universal mechanismin pseudotumor cerebri of varying etiologies.Neurology 1996; 46:198–202.Kelman SE, Heaps R, Wolf A, Elman MJ. Optic nerve decompressionsurgery improves visual function in patients withpseudotumor cerebri. Neurosurg 1992; 30:391–5.Keltner J. Optic nerve sheath decompression: how does itwork? Has its time come? Arch Ophthalmol 1988;106:1378–83.Konomi H, Imai M, Nihei K, Kamoshita S, Tada H.Indomethacin causing pseudotumor cerebri in Bartter’ssyndrome. N Eng J Med 1978; 298:855.Kupersmith MJ, Gamell L, Turbin R, Peck V, Spiegel P, WallM. Effects of weight loss on the course of idiopathicintracranial hypertension in women. Neurology 1998;50:1094–8.Marcelis J, Silberstein SD. Idiopathic intracranial hypertensionwithout papilledema. Arch Neurol 1991; 48:392–9.Mathew NT, Ravinshankar K, Sanin LC. Coexistence ofmigraine and idiopathic intracranial hypertension withoutpapilledema. Neurology 1996; 46:1226–30.Nicolas J, Ramadan NM. Idiopathic intracranial hypertension.In: Neurobase. La Jolla: Arbor Pubs 1998.Radhkrishnan K, Ahlskog JE, Cross SA, Kurland LT, O’FallonWM. Idiopathic intracranial hypertension (pseudotumorcerebri). Descriptive epidemiology in Rochester, Minnesota,1976 to 1990. Arch Neurol 1993; 50:78–80.Ramadan NM. Headache related to increased intracranialpressure and intracranial hypotension. Curr OpinionNeurol 1996; 9:214–8.Sergott RC, Savino PJ, Bosley TM. Modified optic nerve sheathdecompression provides long-term visual improvement forpseudotumor cerebri. Arch Ophthalmol 1988; 106:1384–90.Spector RH, Carlisle J. Pseudotumor cerebri caused by a syntheticvitamin A preparation. Neurology 1984; 34:1509–11.Spence JD, Amacher AL, Willis NR. Benign intracranial hypertensionwithout papilledema: role of 24 hour cerebrospinalfluid pressure monitoring in diagnosis and management.Neurosurgery 1980; 7:326–36.Sugerman HJ, Felton WL, Sismanis A, Kellum JM, DeMariaEJ, and Sugerman EL. Gastric surgery for pseudotumorcerebri associated with severe obesity. Ann Surg 1999;229:634–40.Wall M. The headache profile of idiopathic intracranial hypertension.Cephalalgia 1990; 10:331–5.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 85Wall M. Idiopathic intracranial hypertension. Neurol Clin1991; 9:73–95.Wall M, George D. Idiopathic intracranial hypertension. Brain1991; 114:155–80.Wall M, George D. Idiopathic intracranial hypertension: aprospective study of 50 patients. Brain 1991; 114:155–80.Wall M, White WN. Asymmetric papilledema in idiopathicintracranial hypertension: prospective interocular comparisonof sensory visual function. Inv Ophthalmol Vis Sci1998; 39:134–42.Wang SJ, Silberstein SD, Patterson S, Young WB. Idiopathicintracranial hypertension without papilledema: A casecontrolstudy in a headache center. Neurology 1998;51:245–9.Weisberg LA. Benign intracranial hypertension. Medicine1975; 54:197–207.7.2.1 Post-dural (post-lumbar) puncture headacheBruera OC, Bonamico L, Giglio JA, Sinay V, Leston JA,Figuerola M de L. Intracranial hypotension: the non specificnature of MRI findings. Headache 2000; 40:848–52.Camann WR, Murray S, Mushlin PS, Lambert DH. Effects oforal caffeine on postural puncture headache. A doubleblind,placebo-controlled trial. Anesth Analg 1990; 70:181–4.Christoforidis GA, Mehta BA, Landi JL, Czarnecki EJ,Piaskowski RA. Spontaneous intracranial hypotension:report of four cases and review of the literature. Neuroradiology1998; 40:636–43.Chung SJ, Kim JS, Lee M. Syndrome of cerebral fluid hypovolemia.Clinical and imaging features and outcome. Neurology2000; 55:1321–7.Connelly NR, Parker RK, Rahimi A, Gibson CS. Sumatriptanin patients in postdural puncture headache. Headache 2000;40:316–9.Dieterich M, Brandt T. Is obligatory bed rest after lumbarpuncture obsolete. Europ Arch Psychiatr Neurol Sci 1985;235:71–5.Evans RW, Armon C, Frohman EM, Goodin GS. Assessment:prevention of post-lumbar puncture headaches. Report ofthe Therapeutics and Technology Assessment Subcommiteeof the American Academy of Neurology. Neurology 2000;55:909–14.Fischman RA, Dillon WP. Dural enhancement and cerebraldisplacement secondary to intracranial hypotension. Neurology1993; 43:609–11.Flaatten H, Kräkenes J, Vedeler C. Post-dural puncture relatedcomplications after diagnostic lumbar puncture, myelographyand spinal anaesthesia. Acta Neurol Scand 1998; 98:445–51.Hochman S, Naidich TP, Kobetz SA, Fernandez-Maitin A.Spontaneous intracranial hypotension with pachymeningealenhancement on MRI. Neurology 1992; 42:1628–30.Kovanen J, Sulkava R. Duration of postural headache afterlumbar puncture: effect of needle size. Headache 1986; 26:224–6.Levine DN, Rapalino O. The pathophysiology of lumbarpuncture headache. J Neurol Sci 2001; 192:1–8.Mokri B, Atkinson JLD, Piepgras DG. Absent headachedespite CSF volume depletion (intraranial hypotension).Neurology 2000; 55:1722–4.Mokri B, Maher CO, Sencavoka D. Spontaneous CSF leaks:underlying disorder of connective tissue. Neurology 2002;58:814–6.Mokri B, Parisi JE, Scheithauer BW, Piepgras DG, Miller GM.Meningeal biopsy in intracranial hypotension: Menigealenhancement on MRI. Neurology 1995; 45:1801–7.Mokri B, Posner JB. Spontaneous intracranial hypotension.The broadening spectrum of CSF leaks. Neurology 2000;55:1771–2.Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranialhypotension to cerebrospinal fluid hypovolemia – evolutionof a concept. Mayo Clin Proc 1999; 74:1113–23.Morrow JI. Failure of oblique needle insertion to prevent postlumbarpuncture headache. Arch Neurol 1987; 44:795–5.O’Carroll CP, Brant-Zawadzki M The syndrome of spontaneousintracranial hypotension. Cephalalgia 1999; 19:80–7.Olsen KS. Epidural blood patch in the treatment of postlumbarpuncture headache. Pain 1987; 30:293–301.Rabin B, Roychowdhury S, Meyer JR, Cohen BA, LaPat KD,Russel EJ. Spontaneous intracranial hypotension: spinalMRI findings. AJNR Am J Neuroradiol 1998; 19:1034–9.Rando TA, Fishman RA. Spontaneous intracranial hypotension:report of two cases and review of the literature. Neurology1992; 42:481–7.Raskin NH. Lumbar puncture headache: a review. Headache1990; 30:197–200.Schievink WI, Morreale VM, Atkinson JLD, Meyer FB, PiepgrasDG, Ebersold MJ. Surgical treatment of spontaneousspinal cerebrospinal fluid leaks. J Neurosurg 1998; 88:243–6.Schwalbe SS, Schiffmiller MW, Marx GF. Theophylline forpost-dural puncture headache. Anaesthesiology 1991;75:A1081.Sencavoka D, Mokri B, McClelland RL. The efficacy ofepidural blood patch in spontaneous CSF leaks. Neurology2001; 57:1921–3.Strupp M, Brandt T, Müller A. Incidence of post-lumbar puncturesyndrome reduced by reinserting the stylet: a randomizedprospective study of 600 patients. J Neurol 1998;245:589–92.Thomas SR, Jamieson DRS, Muir KW. Randomised controlledtrial of atraumatic versus standard needles for diagnosticlumbar puncture. BMJ 2000; 321:986–90.Vilming ST, Ellertsen B, Troland K, Schrader H, Monstad I.MMPI profiles in post-lumbar puncture headache. ActaNeurol Scand 1997; 95:184–8.Vilming ST, Kloster R. Post-lumbar puncture headache: clinicalfeatures and suggestions for diagnostic criteria. Cephalalgia1997; 17:778–84.Vilming ST, Kloster R. The time course of post-lumbar punctureheadache. Headache 1998; 18:97–100.7.3.1 Headache attributed to neurosarcoidosis,7.3.2 Headache attributed to aseptic (noninfectious)meningitisAiniala H, Loukkoa J, Peltola J, Korpela Migraine, HietaharjuA. The prevalence of neuropsychiatric syndromes in systemiclupus erythematosus. Neurology 2001; 57:496–500.Al-Fahad SA, Al-Araji AH. Neuro-Behcet’s disease in Iraq: astudy of 40 patients. J Neurol Sci 1999; 170:105–11.Bachmeyer C, de la Blanchardiere A, Lepercq J, Dhote R,Grateau G, Detilleux M, Tournaire M, Christoforov B.Diagnosing headache type (IHS 2003)86 ICHD-IIRecurring episodes of meningitis (Mollaret’s meningitis)with one showing an association with herpes simplex virustype 2. J Infect 1996; 32:247–8.Bakheit AM, Kennedy PG, Graham DI, More JR. Idiopathicgranulomatous meningitis. J Neurol Neurosurg Psychiat1989; 52:1286–9.Bartleson JD, Swanson JW, Whisnant JP. A migrainous syndromewith cerebrospinal fluid pleocytosis. Neurology1981; 31:1257–62.Berg MJ, Williams LS. The transient syndrome of headachewith neurologic deficits and CSF lymphocytosis. Neurology1995; 45:1648–54.Brey RL, Gharavi AE, Lockshin MD. Neurologic complicationsof antiphospholipid antibodies. Rheum Dis ClinNorth Am 1993; 19:833–50.Cooper SD, Brady MB, Williams JP, Pilgreen KL, Harp DL,Weissmann JR. Neurosarcoidosis: evaluation using computedtomography and magnetic resonance imaging. JComput Tomogr 1988; 12:96–9.Cohen BA, Rowley AH, Long CM. Herpes simplex type 2 ina patient with Mollaret’s meningitis:meningitis:demonstrationby polymerase chain reaction. Ann Neurol 1994; 35:112–6.Chapelon C, Ziza JM, Piette JC, Levy Y, Raguin G, Wechsler Bet al. Neurosarcoidosis: signs, course and treatment in 35confirmed cases. Medicine (Baltimore) 1990; 69:261–76.Farah, Al-Shubaili A, Montaser A, Hussein JM, Malaviya AN,Mukhtar M, Al-Shayeb A, Khuraibet AJ, Khan R, TronteljJV. Behcet’s syndrome: a report of 41 patients with emphasison neurological manifestations. J Neurol Neurosurg Psychiat1998; 64:382–4.Glanz BI, Venkatesan A, Schur PH, Lew RA, Khoshbin S.Prevalence of migraine in patients with systemic lupus erythematosus.Headache 2001; 41:285–9.Gullapalli D, Phillips LH 2nd. Neurologic manifestations ofsarcoidosis. Neurol Clin 2002; 20:59–83.Hollinger P, Sturzenegger M. Mathis J, Schroth G, Hess CW.Acute disseminated encephalomyelitis in adults: a reappraisalof clinical CSF, EEG and MRI findings. J Neurol2002; 249:320–9.Jensenius M, Myrvang B, Storvold G, Bucher A, Hellum KB,Bruu Al. Herpes simplex virus type 2 DNA detected in cerebrospinalfluid of 9 patients with Mollaret’s meningitis.Acta Neurol Scand 1998; 98:209–12.Meng MV, St Lezin M. Trimethoprim-sulfamethoxazoleinduced recurrent aspetic meningitis. J Urol 2000; 164:1664–5.Moris G, Garcia-Monco JC. The challenge of drug-inducedaseptic meningitis. Arch Intern Med 1999; 159:1185–94.Nicolas J, Ramadan NM. Idiopathic intracranial hypertension.In: Neurobase. La Jolla: Arbor Pubs 1998.Nowak DA, Widenka DC. Neurosarcoidosis: a review of itsintracranial manifestation. J Neurol 2001; 248:363–72.Omdal R, Waterloo K, Koldingsnes W, Husby G, Mellgren SI.Somatic and psychological features of headache in systemiclupus erythematosus. J Rheumatol 2001; 28:772–9.Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-HagenlocherB. Acute disseminated encephalomyelitis; a followupstudy of 40 adult patients. Neurology 2001; 56:1313–8.Seaton France AJ. Recurrent aseptic meningitis following nonsteroidalanti-inflammatory drugs – a reminder. PostgradMed J 1999; 75:771–2.Sharma OP. Neurosarcoidosis. Chest 1991; 100:301–2.Sharma OP. Neurosarcoidosis: a personal perspective basedon the study of 37 patient. Chest 1997; 112:220–8.Stamboulis E, Spengos M, Rombos A, Haidemenos A. Asepticinflammatory meningeal reaction manifesting as a migrainoussyndrome. Headache 1987; 27:439–41.Sylaja PN, Cherian PJ, Das CK, Raddhakrishnan W, RadhakrishnanK. Idiopathic hypertrophic cranial pachymeningitis.Neurol India 2002; 50:53–9.Tselis A. Acute disseminated encephalomyelitis. Curr TreatOptions Neurol 2001; 3:537–42.Vinas FC, Rengachary S. Diagnosis and management of neurosarcoidosis.J Clin Neurosci 2001; 8:505–13.Von Storch TJC, Merritt HH. The cerebrospinal fluid duringand between attacks of migraine headaches. Am J Med Sci1935; 190:226–31.Walker A, Tyor W. Neurosarcoidosis. Curr Treat OptionsNeurol 2001; 3:529–35.7.6 Headache attributed to epileptic seizureBernasconi A, Andermann F, Bernasconi N, Reutens DC,Dubeau F. Lateralizing value of peri-ictal headache: Astudyof 100 patients with partial epilepsy. Neurology 2000;56:130–2.Isler H, Wieser HG, Egli M. Hemicrania epileptica: synchronousipsilateral ictal headache with migraine features. In:Andermann F, Lugaresi E, eds. Migraine and epilepsy.Boston, MA: Butterworth 1987; pp. 249–63.Laplante P, Saint–Hilaire JM, Bouvier G. Headache as anepileptic manifestation. Neurology 1983; 33:1493–5.Leniger T, Isbruch K, Von den Driesch S, Diener HC, HufnagelA. Seizure-associated headache in epilepsy. Epilepsia 2001;42:1176–9.Lennox WG, Lennox MA. Epilepsy and related disorders.Boston: Little Brown 1960.Marks DA, Ehrenberg BL. Migraine-related seizures in adultswith epilepsy, with EEG correlation. Neurology 1993;43:2476–83.Panayiotopoulos CP, Ahmed Sharoqi I, Agathonikou A.Occipital seizures imitating migraine aura. J R Soc Med1997; 90:255–7.Schon F, Blau JN. Post-epileptic headache and migraine. JNeurol Neurosurg Psychiat 1987; 50:1148–52.Sturzenegger MH, Meienberg O. Basilar artery migraine: afollow-up study of 82 cases. Headache 1985; 25:408–15.Terzano MG, Parrino L, Pietrini V et al. Migraine-epilepsysyndrome: intercalated seizures in benign occipitalepilepsy. In: Andermann F, Beaumanoir A, Mira L, eds.Occipital seizures and epilepsies in children. London: JohnLibbey 1993; pp. 93–9.Young GB, Blume WT. Painful epileptic seizures. Brain 1983;106:537–54.7.7 Headache attributed to Chiari malformationtype I (CM1)Milhorat TH, Chou MW, Trinidad EM et al. Chiari I malformationredefined: clinical and radiographic findings for 364symptomatic patients. Neurosurgery 1999; 44:1005–17.Nohria V, Oakes WJ. Chiari I malformation: a review of 43patients. Pediatr Neurosurg 1990–91; 16:222–7.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 87Nohria V, Oakes WJ. Chiari headaches. Neurology 1993;43:1272.Pascual J, Iglesias F, Oterino A et al. Cough, exertional, andsexual headaches: an analysis of 72 benign and symptomaticcases. Neurology 1996; 46:1520–4.Pascual J, Oterino A, Berciano J. Headache in type I Chiarimalformation. Neurology 1992; 42:1519–21.Stovner LJ. Headache associated with the Chiari malformation.Headache 1993; 33:175–81.7.8 Syndrome of transient headache andneurologic deficits with cerebrospinal fluidlymphocytosisBartleson JD, Swanson JW, Whisnant JP. A migrainous syndromewith cerebrospinal fluid pleocytosis. Neurology1981; 31:1257–62.Berg MJ, Williams LS. The transient syndrome of headachewith neurologic deficits and CSF lymphocytosis (review).Neurology 1995; 45:1648–54.Gomez-Aranda F, Canadillas F, Marti-Masso JF, Diez-TejedorE et al. Pseudomigraine with temporary neurological symptomsand lymphocytic pleocytosis: A report of fifty cases.Brain 1997; 120:1105–13.Diagnosing headache type (IHS 2003)88 ICHD-II8. Headache attributed to a substance or itswithdrawal8.1 Headache induced by acute substance use orexposure8.1.1 Nitric oxide (NO) donor-inducedheadache8.1.1.1 Immediate NO donor-inducedheadache8.1.1.2 Delayed NO donor-headache8.1.2 Phosphodiesterase (PDE) inhibitorinducedheadache8.1.3 Carbon monoxide-induced headache8.1.4 Alcohol-induced headache8.1.4.1 Immediate alcohol-inducedheadache8.1.4.2 Delayed alcohol-inducedheadache8.1.5 Headache induced by food componentsand additives8.1.5.1 Monosodium glutamate-inducedheadache8.1.6 Cocaine-induced headache8.1.7 Cannabis-induced headache8.1.8 Histamine-induced headache8.1.8.1 Immediate histamine-inducedheadache8.1.8.2 Delayed histamine-inducedheadache8.1.9 Calcitonin gene-related peptide (CGRP)-induced headache8.1.9.1 Immediate CGRP-inducedheadache8.1.9.2 Delayed CGRP-inducedheadache8.1.10 Headache as an acute adverse eventattributed to medication used for otherindications8.1.11 Headache induced by other acutesubstance use or exposure8.2 Medication-overuse headache (MOH)8.2.1 Ergotamine-overuse headache8.2.2 Triptan-overuse headache8.2.3 Analgesic-overuse headache8.2.4 Opioid-overuse headache8.2.5 Combination medication-overuseheadache8.2.6 Headache attributed to other medicationoveruse8.2.7 Probable medication-overuse headache8.3 Headache as an adverse event attributed tochronic medication8.3.1 Exogenous hormone-induced headache8.4 Headache attributed to substance withdrawal8.4.1 Caffeine-withdrawal headache8.4.2 Opioid-withdrawal headache8.4.3 Oestrogen-withdrawal headache8.4.4 Headache attributed to withdrawal fromchronic use of other substancesCoded elsewhere:7.1.2 Headache attributed to intracranial hypertensionsecondary to metabolic, toxic or hormonal causes, 7.3.2Headache attributed to aseptic (non-infectious) meningitis,10.3.6 Headache attributed to acute pressor responseto an exogenous agent.General commentPrimary or secondary headache or both?When a new headache occurs for the first time inclose temporal relation to substance exposure, it iscoded as a secondary headache attributed to thesubstance. This is also true if the headache has thecharacteristics of migraine, tension-type headacheor cluster headache. When a pre-existing primaryheadache is made worse in close temporal relationto substance exposure, there are two possibilities,and judgment is required. The patient can either begiven only the diagnosis of the pre-existing primaryheadache or be given both this diagnosis and thediagnosis of headache attributed to the substance.Factors that support adding the latter diagnosisare: a very close temporal relation to the substanceexposure, a marked worsening of the pre-existingheadache, very good evidence that the substancecan aggravate the primary headache and, finally,improvement or resolution of the headache after terminationof effect of the substance.Definite, probable or chronic?A diagnosis of Headache attributed to a substanceusually becomes definite only when the headacheresolves or greatly improves after termination ofexposure to the substance. When exposure to asubstance ceases but headache does not resolve ormarkedly improve after 3 months, a diagnosis of A8.5Chronic post-substance exposure headache, described inthe appendix, may be considered. However, suchheadaches have not been documented and the criteriaare proposed only for research purposes.In the particular case of 8.2 Medication-overuseheadache, a period of 2 months after cessation ofoveruse is stipulated in which improvement mustoccur if the diagnosis is to be definite. Prior to cessation,or pending improvement within 2 monthsafter cessation, the diagnosis 8.2.7 Probable medication-overuse headache should be applied. If improve-Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 89ment does not then occur within the 2 months, thisdiagnosis must be discarded.IntroductionMigraineurs are physiologically and perhaps psychologicallyhyper-responsive to a variety of internaland external stimuli. Alcohol, food and foodadditives and chemical and drug ingestion and withdrawalhave all been reported to provoke or activatemigraine in susceptible individuals. The associationis often based on anecdotal data and reports ofadverse drug reactions.The fact that these stimuli are associated withheadache does not prove causation or eliminate theneed to consider other aetiologies. Because commonevents happen commonly, the association betweena headache and an exposure to a substance may bemere coincidence. Headache can occur just on thebasis of chance. Headache can be a symptom of asystemic disease, and drugs given to treat such acondition will be associated with headache. In acutemigraine drug trials, headache, as well as associatedsymptoms, is listed as an adverse drug reactiondespite that it is a symptom of the treated disorderand not the result of treatment. Some disorders maypredispose to substance-related headache. Alone,neither the drug nor the condition would produceheadache. A nonsteroidal anti-inflammatory drugmay produce headache by inducing aseptic meningitisin susceptible individuals.Finally, some acute or chronic substance exposureshave been proven to be causally related to headache.8.1 Headache induced by acute substance use orexposureCoded elsewhere:10.3.6 Headache attributed to acute pressor response to anexogenous agent.IntroductionThis group of headache disorders can be caused 1)by an unwanted effect of a toxic substance, 2) by anunwanted effect of a substance in normal therapeuticuse and 3) in experimental studies.Substances that cause headache through theirtoxic effects, such as carbon monoxide, cannot bestudied experimentally and the causal relationshipbetween exposure and headache has therefore to bedemonstrated in clinical cases where the substancehas been used accidentally or for suicide attempt.Headache as a side effect has been recorded withmany drugs, often as just a reflection of the very highprevalence of headache. Only when it occurs moreoften after active drug than after placebo in doubleblindcontrolled trials can headache be regarded asa true side effect. The double-blind design can alsobe used experimentally to study the relationshipbetween drug effects and headache. In some cases,for example NO donors, such studies have led to adeeper understanding of the involvement of neurotransmittermechanisms in primary headaches. Anumber of substances such as NO donors and histamineinduce an immediate headache in normal volunteersand in migraineurs. However, it is now clearthat sufferers of primary headache also develop adelayed headache one to several hours after theinducing substance has been cleared from the blood.Knowing the potential headache-inducing effectsof substances in clinical use is important in order tolabel these substances appropriately. In general,migraine sufferers are much more susceptible tosuch headaches than other individuals and the samemay be true for sufferers of chronic tension-typeheadache, episodic tension-type headache andcluster headache during cluster periods.Paradoxically, the headache encountered by mostpeople after heavy alcohol use may be a positivefeature because it helps avoid excessive drinking.Combinations such as alcohol and disulfiram maycause headache when individual agents might not.8.1.1 Nitric oxide (NO) donor-induced headache8.1.1.1 Immediate NO donor-induced headacheDiagnosing headache type previously used terms:Nitroglycerine headache, dynamite headache, hotdog headacheDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. frontotemporal location3. pulsating quality4. aggravated by physical activityB. Absorption of a NO donorC. Headache develops within 10 minutes afterabsorption of NO donorD. Headache resolves within 1 hour after release ofNO has ended8.1.1.2 Delayed NO donor-induced headacheDiagnosing headache type diagnostic criteria:A. Headache, in a person who suffers from primaryheadache, with the characteristics of that primaryheadache type1 and fulfilling criteria C and DDiagnosing headache type (IHS 2003)90 ICHD-IIB. Absorption of a NO donorC. Headache develops after NO is cleared from theblood2D. Headache resolves within 72 hours after singleexposureNotes:1. Normal subjects rarely develop delayed NOdonor-induced headache whilst migraineursdevelop an attack of migraine without aura,tension-type headache sufferers develop atension-type headache and cluster headache sufferersdevelop a cluster headache attack.2. Migraine and tension-type headache developafter a mean of 5–6 hours, cluster headache typicallyafter 1–2 hours.Diagnosing headache type comments:The headache is typically bilateral, pulsating andfrontotemporal in location.All NO donors (eg, amyl nitrate, erythrityl tetranitrate,glyceryl trinitrate [GTN], isosorbide mono- ordinitrate, sodium nitroprusside, mannitol hexanitrate,pentaerythrityl tetranitrate) can causeheadache of this subtype particularly in personswith migraine. GTN is the best studied substance. Itreliably induces headache in most normal individualsand migraine sufferers develop a more severeimmediate headache than non-migraine sufferers.GTN can also cause a delayed headache in migrainesufferers which fulfils the diagnostic criteria for 1.1Migraine without aura, even in patients whose spontaneousmigraine attacks are with aura. In peoplewith chronic tension-type headache, GTN has beenshown to induce a delayed headache which has thecharacteristics of tension-type headache. It is notknown if it has the same effect in sufferers ofepisodic tension-type headache. Cluster headachesufferers do not develop delayed headache outsidecluster periods but, during a cluster period, GTNfairly reliably induces a cluster headache attackusually occurring 1–2 hours after intake. Thedelayed headache in those with migraine or tensiontypeheadache occurs at variable times but onaverage 5–6 hours after exposure.Headache is well known as a side effect of therapeuticuse of nitroglycerine and other NO donors.With chronic use tolerance develops within a week,and GTN-induced headache disappears in mostpatients within that time. With intermittent useheadache continues, and may be severe enough tocompromise the use of NO donors for angina. Mostheart patients are, however, male and beyondmiddle age, which probably explains why theproblem is not of greater magnitude.Other NO donors have been much less studied butavailable evidence suggests that they too mayproduce headache. Isosorbide mononitrate has beenthe subject of one formal double-blind placebocontrolledstudy and causes a much longer-lastingheadache than GTN owing to its slow release of NO.8.1.2 Phosphodiesterase (PDE) inhibitor-inducedheadacheDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. frontotemporal location3. pulsating quality4. aggravated by physical activityB. A single dose of a phosphodiesterase inhibitorhas been givenC. Headache develops within 5 hours of PDEinhibitor intakeD. Headache resolves within 72 hoursDiagnosing headache type comment:PDEs are a large family of enzymes that break downcyclic nucleotides cGMP and cAMP. When PDEs areinhibited, the levels of cGMP and/or cAMP thereforeincrease. PDE-5 inhibitors sildenafil and dipyridamoleare the only formally studied compounds inthis group. The headache, unlike GTN-inducedheadache, is monophasic. In normal volunteers ithas the characteristics of tension-type headache butin migraine sufferers it has the characteristics ofmigraine without aura. Headache has been noted asa side effect of sildenafil in clinical trials but onlyrecent experimental studies have shown that, inyoung persons – especially females – the side effectoccurs in a majority of subjects and in migrainepatients sildenafil usually induces a migraine attack.Migraine sufferers should be warned of this sideeffect.8.1.3 Carbon monoxide-induced headacheDiagnosing headache type previously used terms:Warehouse workers’ headacheDiagnosing headache type diagnostic criteria:A. Bilateral and/or continuous headache, withquality and intensity that may be related to theseverity of carbon monoxide intoxication1, fulfillingcriteria C and DB. Exposure to carbon monoxide (CO)Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 91C. Headache develops within 12 hours of exposureD. Headache resolves within 72 hours after eliminationof carbon monoxideNote:1. Typically: mild headache without gastrointestinalor neurological symptoms with carboxyhaemoglobinlevels in the range 10–20%; moderatepulsating headache and irritability with levels of20–30%; severe headache with nausea, vomitingand blurred vision with levels of 30–40%.Diagnosing headache type comments:With higher carboxyhaemoglobin levels (>40%)headache is not usually a complaint because ofchanges in consciousness.There are no good studies of the long-term effectsof CO intoxication on headache. Casuistic evidencesuggests the possibility of chronic post-intoxicationheadache.8.1.4 Alcohol-induced headache8.1.4.1 Immediate alcohol-induced headacheDiagnosing headache type previously used terms:Cocktail headacheDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. frontotemporal location3. pulsating quality4. aggravated by physical activityB. Ingestion of beverage containing alcohol1C. Headache develops within 3 hours after ingestionof alcoholic beverageD. Headache resolves within 72 hoursNote:1. The effective dose has not been determined.Diagnosing headache type comment:A few subjects develop headache due to a directeffect of alcohol or alcoholic beverages. This is muchrarer than delayed alcohol-induced headache.8.1.4.2 Delayed alcohol-induced headacheDiagnosing headache type previously used terms:Hangover headacheDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. frontotemporal location3. pulsating quality4. aggravated by physical activityB. Ingestion of a modest amount of alcoholic beverageby a migraine sufferer or an intoxicatingamount by a non-migraine suffererC. Headache develops after blood alcohol leveldeclines or reduces to zeroD. Headache resolves within 72 hoursDiagnosing headache type comment:This is one of the commonest types of headache. Itremains unclear whether, in addition to alcohol,other components of alcoholic beverages play a role.It also remains uncertain whether the mechanism isa delayed response to toxic effects or whether mechanismssimilar to those responsible for delayed NOdonor-induced headache may be involved.The susceptibility to hangover headache of welldiagnosedheadache patients compared with nonheadachesufferers has not been determined. Inmigraine sufferers a migraine attack can be inducedthe next day after modest intake of alcoholic beverages,while non-migraineurs usually need a highintake of alcoholic beverages in order to develop8.1.4.2 Delayed alcohol-induced headache.8.1.5 Headache induced by food components andadditivesDiagnosing headache type previously used terms:Dietary headacheDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. frontotemporal location3. pulsating quality4. aggravated by physical activityB. Ingestion of a minimum dose of food componentor additive1C. Headache develops within 12 hours after substanceintakeD. Headache resolves within 72 hours after singleintakeNote:1. Phenylethylamine, tyramine and aspartame havebeen incriminated but their headache-inducingpotential is not sufficiently validated.Diagnosing headache type (IHS 2003)92 ICHD-II8.1.5.1 Monosodium glutamate-induced headacheDiagnosing headache type previously used terms:Chinese restaurant syndromeDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. frontotemporal location3. aggravated by physical activityB. Ingestion of monosodium glutamate (MSG)C. Headache develops within 1 hour after MSGingestionD. Headache resolves within 72 hours after singleintakeDiagnosing headache type comment:MSG-induced headache is typically dull or burningand non-pulsating, but may be pulsating in migrainesufferers. It is commonly associated with othersymptoms of this syndrome including pressure in thechest, pressure and/or tightness in the face, burningsensations in the chest, neck or shoulders, flushing offace, dizziness and abdominal discomfort.8.1.6 Cocaine-induced headacheDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. frontotemporal location3. pulsating quality4. aggravated by physical activityB. Use of cocaineC. Headache develops within 1 hour after cocaineuseD. Headache resolves within 72 hours after singleuseDiagnosing headache type comment:Headache is a reported side effect of cocaine use. Itis frequent, develops immediately or within onehour after use and is not associated with other symptomsunless there is concomitant stroke or TIA.8.1.7 Cannabis-induced headacheDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. stabbing or pulsating quality3. feeling of pressure in the headB. Use of cannabisC. Headache develops within 12 hours aftercannabis useD. Headache resolves within 72 hours after singleuseDiagnosing headache type comment:Cannabis use is reported to cause headache associatedwith dryness of the mouth, paraesthesias, feelingsof warmth and suffusion of the conjunctivae.8.1.8 Histamine-induced headacheDiagnosing headache type comment:Histamine has been shown to cause an immediateheadache in non-headache sufferers and an immediateas well as a delayed headache in migrainesufferers. The latter fulfils criteria for 1.1 Migrainewithout aura. The headache-inducing property of histaminehas been studied after intravenous administration,after cutaneous administration and afterinhalation: all routes of administration have thesame effect. The mechanism is primarily mediatedvia the H1 receptor because it is almost completelyblocked by mepyramine.8.1.8.1 Immediate histamine-induced headacheDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. frontotemporal location3. pulsating quality4. aggravated by physical activityB. Absorption of histamineC. Headache develops within 10 minutes afterabsorption of histamineD. Headache resolves within 1 hour after absorptionof histamine has ceased8.1.8.2 Delayed histamine-induced headacheDiagnosing headache type diagnostic criteria:A. Headache, in a person who suffers from primaryheadache, with the characteristics of that primaryheadache type1 and fulfilling criteria C and DB. Absorption of histamineC. Headache develops after histamine is clearedfrom the blood2D. Headache resolves within 72 hours after singleexposureNotes:1. Normal subjects rarely develop delayed histamine-induced headache whilst migraineursDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 93develop an attack of migraine without aura,tension-type headache sufferers develop atension-type headache and cluster headache sufferersdevelop a cluster headache attack.2. Migraine and tension-type headache developtypically after 5–6 hours, cluster headache typicallyafter 1–2 hours.8.1.9 Calcitonin gene-related peptide (CGRP)-induced headacheDiagnosing headache type comment:The headache-inducing property of CGRP has beenstudied only in one double-blind controlled trial.There is, however, no doubt that CGRP causes animmediate headache. Delayed migraine attacks wereinduced in 3 out of 10 subjects. Recently, it has beenshown that a CGRP antagonist is effective in theacute treatment of migraine.8.1.9.1 Immediate CGRP-induced headacheDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. frontotemporal location3. pulsating quality4. aggravated by physical activityB. Absorption of CGRPC. Headache develops within 10 minutes afterabsorption of CGRPD. Headache resolves within 1 hour after absorptionof CGRP has ceased8.1.9.2 Delayed CGRP-induced headacheDiagnosing headache type diagnostic criteria:A. Headache, in a person who suffers from primaryheadache, with the characteristics of that primaryheadache type1 and fulfilling criteria C and DB. Absorption of CGRPC. Headache develops after CGRP is cleared fromthe blood2D. Headache resolves within 72 hours after infusionof CGRPNotes:1. Normal subjects rarely develop delayed CGRPinducedheadache whilst migraineurs developan attack of migraine without aura, tension-typeheadache sufferers develop a tension-type headacheand cluster headache sufferers develop acluster headache attack.2. Migraine and tension-type headache developtypically after 5–6 hours, cluster headache typicallyafter 1–2 hours.8.1.10 Headache as an acute adverse eventattributed to medication used for other indicationsDiagnosing headache type diagnostic criteria:A. Headache fulfilling criteria C and DB. Use of a medication for a therapeutic indicationother than headacheC. Headache develops within minutes to hours afteruseD. Headache resolves within 72 hours after cessationof useDiagnosing headache type comments:Headache has been reported after use of a numberof drugs. The following are the most commonlyincriminated: atropine, digitalis, disulfiram,hydralazine, imipramine, nicotine, nifedipine,nimodipine. Alonger list can be found in the appendix(table 1).The headache characteristics are not very welldefined in the literature but most are dull, continuous,diffuse and moderate to severe.8.1.11 Headache induced by other acute substanceuse or exposureDiagnosing headache type diagnostic criteria:A. Headache fulfilling criteria C and DB. Acute use of or other acute exposure to a substanceother than those described aboveC. Headache develops within 12 hours of use orexposureD. Headache resolves within 72 hours after singleuse or exposureDiagnosing headache type comments:Headache has been reported after exposure to anumber of organic and inorganic substances. Thefollowing are the most commonly incriminated substances:Inorganic compounds: arsenic, borate, bromate,chlorate, copper, iodine, lead, lithium, mercury,tolazoline hydrochloride.Organic compounds: alcohols (long-chain), aniline,balsam, camphor, carbon disulfide, carbon tetrachloride,clordecone, EDTA, heptachlor, hydrogensulfide, kerosene, methyl alcohol, methyl bromide,methyl chloride, methyl iodine, naphthalene,organophosphorous compounds (parathion,pyrethrum).Diagnosing headache type (IHS 2003)94 ICHD-IIThe headache characteristics are not very welldefined in the literature but most are dull, diffuse,continuous and moderate to severe.8.2 Medication-overuse headache (MOH)Diagnosing headache type previously used terms:Rebound headache, drug-induced headache, medication-misuse headacheIntroductionThis and the following section deal with headachedisorders associated with chronic substance use orexposure.Medication-overuse headache is an interactionbetween a therapeutic agent used excessively and asusceptible patient. The best example is overuse ofsymptomatic headache drugs causing headache inthe headache-prone patient.By far the most common cause of migraine-likeheadache occurring on ≥15 days per month and of amixed picture of migraine-like and tension-type-likeheadaches on ≥15 days per month is overuse ofsymptomatic migraine drugs and/or analgesics. Ingeneral, overuse is defined in terms of treatmentdays per month. What is crucial is that treatmentoccurs both frequently and regularly, ie, on severaldays each week. For example, if the diagnostic criterionis use on ≥10 days per month, this translatesinto 2–3 treatment days every week. Bunching oftreatment days with long periods without medicationintake, practised by some patients, is much lesslikely to cause medication-overuse headache.Chronic tension-type headache is less often associatedwith medication overuse but, especiallyamongst patients seen in headache centres, episodictension-type headache has commonly become achronic headache through overuse of analgesics.Patients with a pre-existing primary headachewho develop a new type of headache or whosemigraine or tension-type headache is mademarkedly worse during medication overuse shouldbe given both the diagnosis of the pre-existingheadache and the diagnosis of 8.2 Medication-overuseheadache. Furthermore, the headache associated withmedication overuse often has a peculiar patternshifting, even within the same day, from havingmigraine-like characteristics to having those oftension-type headache (ie, a new type of headache).The diagnosis of medication-overuse headache isclinically extremely important because patientsrarely respond to preventative medications whilstoverusing acute medications.8.2.1 Ergotamine-overuse headacheDiagnosing headache type diagnostic criteria:A. Headache present on >15 days/month with atleast one of the following characteristics and fulfillingcriteria C and D:1. bilateral2. pressing/tightening quality3. mild or moderate intensityB. Ergotamine intake on ≥10 days/month on aregular basis for ≥3 monthsC. Headache has developed or markedly worsenedduring ergotamine overuseD. Headache resolves or reverts to its previouspattern within 2 months after discontinuation ofergotamineDiagnosing headache type comment:Bioavailability of ergots is so variable that aminimum dose cannot be defined.8.2.2 Triptan-overuse headacheDiagnosing headache type diagnostic criteria:A. Headache present on >15 days/month with atleast one of the following characteristics and fulfillingcriteria C and D:1. predominantly unilateral2. pulsating quality3. moderate or severe intensity4. aggravated by or causing avoidance of routinephysical activity (eg, walking or climbingstairs)5. associated with at least one of the following:a) nausea and/or vomitingb) photophobia and phonophobiaB. Triptan intake (any formulation) on ≥10 days/month on a regular basis for ≥3 monthsC. Headache frequency has markedly increasedduring triptan overuseD. Headache reverts to its previous pattern within 2months after discontinuation of triptanDiagnosing headache type comment:Triptan overuse may increase migraine frequency tothat of chronic migraine. Evidence suggests that thisoccurs sooner with triptan-overuse than with ergotamine-overuse.8.2.3 Analgesic-overuse headacheDiagnosing headache type diagnostic criteria:A. Headache present on >15 days/month with atleast one of the following characteristics and fulfillingcriteria C and D:Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 951. bilateral2. pressing/tightening (non-pulsating) quality3. mild or moderate intensityB. Intake of simple analgesics on ≥15 days/month1for >3 monthsC. Headache has developed or markedly worsenedduring analgesic overuseD. Headache resolves or reverts to its previouspattern within 2 months after discontinuation ofanalgesicsNote:1. Expert opinion rather than formal evidence suggeststhat use on ≥15 days/month rather than ≥10days/month is needed to induce analgesicoveruseheadache.8.2.4 Opioid-overuse headacheDiagnosing headache type diagnostic criteria:A. Headache present on >15 days/month fulfillingcriteria C and DB. Opioid intake on ≥10 days/month for >3 monthsC. Headache has developed or markedly worsenedduring opioid overuseD. Headache resolves or reverts to its previouspattern within 2 months after discontinuation ofopioidDiagnosing headache type comment:Prospective studies indicate that patients overusingopioids have the highest relapse rate after withdrawaltreatment.8.2.5 Combination medication-overuse headacheDiagnosing headache type diagnostic criteria:A. Headache present on >15 days/month with atleast one of the following characteristics and fulfillingcriteria C and D:1. bilateral2. pressing/tightening (non-pulsating) quality3. mild or moderate intensityB. Intake of combination medications1 on ≥10days/month for >3 monthsC. Headache has developed or markedly worsenedduring combination medication overuseD. Headache resolves or reverts to its previouspattern within 2 months after discontinuation ofcombination medicationNote:1. Combination medications typically implicatedare those containing simple analgesics combinedwith opioids, butalbital and/or caffeine8.2.6 Headache attributed to other medicationoveruseDiagnosing headache type diagnostic criteria:A. Headache present on >15 days/month fulfillingcriteria C and DB. Regular overuse1 for >3 months of a medicationother than those described aboveC. Headache has developed or markedly worsenedduring medication overuseD. Headache resolves or reverts to its previouspattern within 2 months after discontinuation ofoverused medicationNote:1. The definition of overuse in terms of treatmentdays per month is likely to vary with the natureof the medication.8.2.7 Probable medication-overuse headacheDiagnosing headache type diagnostic criteria:A. Headache fulfilling criteria A–C for any one ofthe subforms 8.2.1 to 8.2.6 aboveB. One or other of the following:1. overused medication has not yet been withdrawn2. medication overuse has ceased within the last2 months but headache has not so far resolvedor reverted to its previous patternDiagnosing headache type comments:Codable subforms of 8.2.7 Probable medication-overuseheadache are 8.2.7.1 Probable ergotamine-overuseheadache, 8.2.7.2 Probable triptan-overuse headache,8.2.7.3 Probable analgesic-overuse headache, 8.2.7.4 Probableopioid-overuse headache, 8.2.7.5 Probable combinationmedication-overuse headache and 8.2.7.6 Headacheprobably attributed to other medication overuse.Many patients fulfilling the criteria for 8.2.7 Probablemedication-overuse headache also fulfil criteria foreither 1.6.5 Probable chronic migraine or 2.4.3 Probablechronic tension-type headache. They should be codedfor both until causation is established after withdrawalof the overused medication. Patients with1.6.5 Probable chronic migraine should additionally becoded for the antecedent migraine subtype (usually1.1 Migraine without aura).8.3 Headache as an adverse event attributed to chronicmedicationDiagnosing headache type diagnostic criteria:A. Headache present on >15 days/month fulfillingcriteria C and DDiagnosing headache type (IHS 2003)96 ICHD-IIB. Chronic medication1 for any therapeuticindicationC. Headache develops during medicationD. Headache resolves after discontinuation ofmedication2Notes:1. The definition of dose and duration will vary withthe medication.2. Time for resolution will vary with the medicationbut may be months.Diagnosing headache type comment:Headache can be due to a direct pharmacologicaleffect of medication, such as vasoconstriction producingmalignant hypertension and headache, or toa secondary effect such as drug-induced intracranialhypertension. The latter is a recognised complicationof long-term use of anabolic steroids, amiodarone,lithium carbonate, nalidixic acid, thyroid hormonereplacement, tetracycline or minocycline.8.3.1 Exogenous hormone-induced headacheDiagnosing headache type diagnostic criteria:A. Headache or migraine fulfilling criteria C and DB. Regular use of exogenous hormonesC. Headache or migraine develops or markedlyworsens within 3 months of commencing exogenoushormonesD. Headache or migraine resolves or reverts to itsprevious pattern within 3 months after total discontinuationof exogenous hormonesDiagnosing headache type comments:Regular use of exogenous hormones, typically forcontraception or hormone replacement therapy, canbe associated with increase in frequency or newdevelopment of headache or migraine.When a woman also experiences headache ormigraine associated with exogenous oestrogenwithdrawal,both codes 8.3.1 Exogenous hormoneinducedheadache and 8.4.3 Oestrogen-withdrawalheadache should be used.8.4 Headache attributed to substance withdrawal8.4.1 Caffeine-withdrawal headacheDiagnosing headache type diagnostic criteria:A. Bilateral and/or pulsating headache fulfillingcriteria C and DB. Caffeine consumption of ≥200mg/day for >2weeks, which is interrupted or delayedC. Headache develops within 24 hours after lastcaffeine intake and is relieved within 1 hour by100 mg of caffeineD. Headache resolves within 7 days after totalcaffeine withdrawal8.4.2 Opioid-withdrawal headacheDiagnosing headache type diagnostic criteria:A. Bilateral and/or pulsating headache fulfillingcriteria C and DB. Opioid intake daily for >3 months, which isinterruptedC. Headache develops within 24 hours after lastopioid intakeD. Headache resolves within 7 days after totalopioid withdrawal8.4.3 Oestrogen-withdrawal headacheDiagnosing headache type diagnostic criteria:A. Headache or migraine fulfilling criteria C and DB. Daily use of exogenous oestrogen for ≥3 weeks,which is interruptedC. Headache or migraine develops within 5 daysafter last use of oestrogenD. Headache or migraine resolves within 3 daysDiagnosing headache type comment:Oestrogen-withdrawal following cessation of acourse of exogenous oestrogens (such as during thepill-free interval of combined oral contraceptives orfollowing a course of replacement or supplementaryoestrogen) can induce headache and/or migraine.8.4.4 Headache attributed to withdrawal fromchronic use of other substancesDiagnosing headache type diagnostic criteria:A. Bilateral and/or pulsating headache fulfilling criteriaC and DB. Daily intake of a substance other than thosedescribed above for >3 months, which is interruptedC. Headache develops in close temporal relation towithdrawal of the substanceD. Headache resolves within 3 months after withdrawalDiagnosing headache type comment:It has been suggested, but without sufficient evidence,that withdrawal of the following substancesmay cause headache: corticosteroids, tricyclic antidepressants,selective serotonin reuptake inhibitorsDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 97(SSRIs), non-steroidal anti-inflammatory drugs(NSAIDs).Bibliography8.1 Headache induced by acute substance use orexposureAltura BM, Altura BT, Gebrewold A. Alcohol induced spasmof cerebral blood vessels. J Mental Sci 2000; 104:972–99.Armstrong PJ, Bersten A. Normeperidine toxicity. AnesthAnalg 1986; 65:536–8.Ashina M, Bendtsen L, Jensen R, Olesen J. Nitric oxideinducedheadache in patients with chronic tension-typeheadache. Brain 2000; 123:1830–7.Askew GL, Finelli L, Genese CA, Sorhage FE, Sosin DM, SpitalnyKC. Bouillabaisse: an outbreak of methemoglobinemiain New Jersey in 1992. Pediatrics 1994; 94:381–4.Askmark H, Lundberg PO, Olsson S. Drug related headache.Headache 1989; 29:441–4.Atkins FM. A critical evaluation of clinical trials in adversereactions to foods in adults. J Allergy Clin Immunol 1986;78:174–82.Beck HG, Schulze WH, Suter GM. Carbon monoxide – adomestic hazard. JAMA 1940; 115:1.Bonnet GF, Nepveux P. Migraine due to tyramine. Sem Hop1971; 47:2441–5.Brewerton TD, Murphy DL, Lesem MD, Brandt HA, JimersonDC. Headache responses following m-chlorophenylpiperazinein bulimics and controls. Headache 1992; 32:217–22.Catalano G, Catalano MC, Rodriguez R. Dystonia associatedwith crack cocaine use. South Med J 1997; 90:1050–2.Cleophas TJ, Niemeyer MG, vanderWall EE, vanderMeulen J.Nitrate-induced headache in patients with stable anginapectoris: beneficial effect of starting on a low dose. Angiology1996; 47:679–85.Council of Scientific Affairs. Aspartame: review of safetyissues. JAMA 1985; 254:400–2.Cregler LL, Mark H. Medical complications of cocaine abuse.N Engl J Med 1986; 315:1495–501.Dhopesh V, Maany I, Herring C. The relationship of cocaineto headache in polysubstance abusers. Headache 1991;31:17–9.Dhuna A, Pascual-Leone A, Belgrade M. Cocaine-relatedvascular headaches. J Neurol Neurosurg Psychiat1991;54:803–6.De Marinis M, Janiri L, Agnoli A. Headache in the use andwithdrawal of opiates and other associated substances ofabuse. Headache 1991; 31:159–63.Ekbom K. Nitroglycerin as a provocative agent in clusterheadache. Arch Neurol 1968; 19:487–93.el-Mallakh RS. Marijuana and migraine. Headache 1987;27:442–3.el-Mallakh RS, Kranzler HR, Kamanitz JR. Headaches andpsychoactive substance use. Headache 1991; 31:584–7.Forbes HS, Cobb S, Fremont-smith F. Cerebral edema andheadache following carbon monoxide asphyxia. ArchNeurol Psychiatr 1924; 11:164.Gawin FH. Cocaine addiction: psychology and neurophysiology.Science 1991; 251:1580–6.Ghose K, Carrol JD. Mechanisms of tyramine-inducedmigraine: similarities with dopamine and interactions withdisulfiram and propranolol. Neuropsychiobiol 1984;12:122–6.Gordon CR, Mankuta D, Shupak A, Spitzer O, Doweck I.Recurrent classic migraine attacks following transdermalscopolamine intoxication. Headache 1991; 31:172–4.Gore ME, Salmon PR. Chinese restaurant syndrome: fact orfiction. Lancet 1980; 318:251–2.Hanington E, Harper AM. The role of tyramine in the etiologyof migraine and related studies on the cerebral andintracerebral circulations. Headache 1968; 8:84–97.Hansen HJ, Drewes VM. The nitroglycerine ointment test – adouble-blind examination. Dan Med Bull 1970; 7:226–9.Heckerling PS, Leikin JB, Maturen A, Perkins JT. Predictorsof occult carbon monoxide poisoning in patients withheadache and dizziness. Ann Int Med 1987; 107:174–6.Henderson WR, Raskin NH. ‘Hot dog’ headache: individualsusceptibility to nitrite. Lancet 1972; ii:1162–3.Hirsch AR, Rankin KM, Panelli PP. Trichloroethylene exposureand headache. Headache Quarterly 1996; 7:126–38.Horowitz LD, Herman MV, Gorlin R. Clinical response tonitroglycerine as a diagnostic test for coronary arterydisease. Am J Cardiol 1972; 29:149–53.Iversen HK, Nielsen TM, Olesen J, Tfelt-Hansen P. Intravenousnitroglycerin as an experimental model of vascularheadache. Basic characteristics. Pain 1989; 38:17–24.Kenney RA, Tidball CS. Human susceptibility to oralmonosodium 1-glutamate. Am J Clin Nutr 1972; 25:140–6.Kerr GR, Lee MW, elLozy M, McGandy R, Stare F. Prevalenceof the ‘Chinese restaurant syndrome’. J Am Diet Assoc 1979;75:29–33.Krabbe AA, Olesen J. Headache provocation by continuousintravenous infusion of histamine, clinical results andreceptor mechanisms. Pain 1980; 8:253–9.Larkin JM, Brahos GJ, Moylin JA. Treatment of carbon monoxidepoisoning: prognostic factors. J Trauma 1976; 16:111.Lassen LH, Thomsen LL, Olesen J. Histamine induces migrainevia the H receptor. Support for the NO-hypothesis ofmigraine. Neuroreport 1995; 6: 1475–9.Leon AS, Hunninghake DB, Bell C, Rassin DK, Tephly TR.Safety of long-term doses of aspartame. Arch Int Med 1989;149:2318–24.Leone M, Attanasio A, Croci D, Filippini G, D’Amico D,Grazzi L, Nespolo A, Bussone G. The serotonergic agent mchlorophenylpiperazineinduced migraine attacks: a controlledstudy. Neurology 2000; 55:136–9.Levine SR, Brust JC, Futrell N, et al. Cerebrovascular complicationsof the use of the crack form of alkaloidal cocaine. NEngl J Med 1990; 323:699–704.Lipton RB, Kwong CM, Solomon S. Headaches in hospitalizedcocaine users. Headache 1989; 29:225–8.Lowenstein DH, Massa SM, Rowbotham MC et al. Acute neurologicand psychiatric complications associated withcocaine abuse. Am J Med 1987; 83:841–6.Luthy J, Schlatter C. Biogenic amines in food: effects of histamine,tyramine and phenylethylamine in the human. ZLebensm Unters Forsch 1983; 177:439–43.McCullock J, Harper AM. Phenylethylamine and the cerebralcirculation. In Current concepts in migraine research (McCullockJ, Harper AM, eds.). Raven Press, New York. 1978, pp.85–8.Diagnosing headache type (IHS 2003)98 ICHD-IIMeigs JL, Hughes JP. Acute carbon monoxide poisoning: ananalysis of 105 cases. AMAArch Ind Hygiene Occupat Med1952; 6:344–56.Merkel PA, Koroshetz WJ, Irizarry MC, Cudkovicz ME.Cocaine-associated cerebral vasculitis. Semin ArthritisRheum 1995; 25:172–83.Merrit JE, Williams PB. Vasospasm contributes tomonosodium glutamate-induced headache. Headache1990; 30:575–80.Mitchell JD. Clinical neurotoxicology: an introduction. InHandbook of Clinical Neurology (DeWolff FA, ed.). ElsevierScience, 1995, pp. 1–22.Moffet AM, Swash M, Scott DF. Effect of chocolate inmigraine: a double-blind study. J Neurol Neurosurg Psychiatry1974; 37:445–8.Monteiro JM. Headache associated with single use of substances.In The headaches (Olesen J, Tfelt-Hansen P, WelchKMA, eds.). Raven Press, Limited, New York. 1993, pp.715–20.Monteiro JM, Dahlof CG. Single use of substances. In TheHeadaches (Olesen J, Tfelt-Hansen P, Welch KM, eds.). LippincottWilliams & Wilkins, Philadelphia. 2000, pp. 861–9.Murphree AB, Greenberg LA, Carrol RB. Neuropharmacologiceffects of substances other than ethanol in alcoholicbeverages. Fed Proc 1967; 26:1468–73.Norton S. Toxicology of the central nervous system. In Toxicology(Casarett LJ, Doult J, eds.). MacMillan, New York.1975, pp. 1019–35.Ogata S, Hosoi T, Saji H. Studies on acute alcohol intoxication.Japanese Journal of Studies of Alcohol 1966; 1:67–79.Peters GA. Migraine: diagnosis and treatment with emphasison the migraine-tension headache, provocative tests anduse of rectal suppositories. Proc Mayo Clin 1953; 28:673–86.Reif-Lehrer L. A questionnaire study of the prevalence ofChinese restaurant syndrome. Fed Proc 1977; 36:1617–23.Sabatini U, Rascol O, Rascol A, Montastruc JL. Migraineattacks induced by subcutaneous apomorphine in twomigrainous Parkinson Ian patients. Clin Neuropharmacol1990; 13:264–7.Satel SL, Gawin FH. Migraine-like headache and cocaine use.JAMA 1989; 261:2995–6.Schamburg HH, Byck R, Gerstl R, Mashman JH. MonosodiumL-glutamate: its pharmacology and role in the Chineserestaurant syndrome. Science 1969; 163:826–8.Scher W, Scher BM. A possible role for nitric oxide in glutamate(MSG)-induced Chinese restaurant syndrome, glutamateinduced asthma, ‘hot-dog headache’, pugilisticAlzheimer’s disease, and other disorders. Med Hypotheses1992; 38:185–8.Schiffmann SS, Buckley CE, Sampson HA et al. Aspartameand susceptibility to headache. N Engl J Med 1987;317:1181–5.Schnitker MT, Schnitker MA. Clinical notes, suggestions andnew instruments. JAMA 1947; 135:89.Schwartz AM. The cause, relief and prevention of headachearising from contact with dynamite. N Engl J Med 1946;235:541–4.Scopp AL. MSG and hydrolyzed vegetable protein inducedheadache review and case studies. Headache 1991;31:107–10.Seltzer S. Foods and drug combinations, responsible for headand neck pain. Cephalalgia 1982; 2:111–24.Shaw SW, Johnson RH, Keogh HG. Oral tyramine in dietarymigraine sufferers. In Current concepts in migraine research(Shaw SW, Johnson RH, Keogh HG, eds.). Raven Press, NewYork. 1978, pp. 31–9.Shively M, Riegel B. Effect of nitroglycerin ointment placementon headache and flushing in health subjects. Int J NursStud 1991; 28:153–61.Sicuteri F, Bene ED, Poggioni M, Bonazzi A. Unmasking latentdysnociception in healthy subjects. Headache 1987;27:180–5.Smith I, Kellow AH, Hanington E. Clinical and biochemicalcorrelation between tyramine and migraine headache.Headache 1970; 10:43–52.Tarasoff L, Kelly MF. Monosodium L-glutamate: a doubleblindstudy and review. Food Chem Toxicol 1993;31:1019–35.Thomsen LL, Kruse C, Iversen HK, Olesen J. A Nitric oxidedonor triggers genuine migraine attacks. Eur J Neurol 1994;1:71–80.Towers CV, Pircon RA, Nageotte MP, Porto M, Garite TJ.Cocaine intoxication presenting as preeclampsia andeclampsia. Ob Gyn 1993; 81:545–7.Trelles L, Jeri R. Central nervous system stimulants: cocaine,amphetamine, nicotine. In Handbook of clinical neurology(DeWolff FA, ed.). New York: Elsevier Science, 1995, pp.251–7.Wallgreen H, Barry A. Drug actions in relation to alcoholeffects. In: Action of alcohol. New York: Elsevier, 1970, pp.621–714.Wilson J. Cyanogenic glycosides. Ch. 3 in Handbook of clinicalneurology, vol. 65 (DeWolff FA, ed.). New York: ElsevierScience, 1995, pp. 25–34.Varon J, Marik PE, Fromm RE, Gueler A. Carbon MonoxidePoisoning: a review for clinicians. J Emerg Med 1999;17:87–93.Yang WH, Drouin MA, Herbert M, Mao Y, Kursh J. Themonosodium glutamate symptom complex: assessment ina double blind, placebo controlled, randomized study. JAllergy Clin Immunol 1997; 99:757–62.8.2 Medication-overuse headacheAla-Hurula V, Myllyla V, Hokkanen E. Ergotamine abuse:results of ergotamine discontinuation with special referenceto the plasma concentrations. Cephalalgia 1982; 2:189–95.Ala-Hurula V, Myllyla V, Hokkanen E, Tokola O. Tolfenamicacid and ergotamine abuse. Headache 1981; 21:240–2.Allgulander C. History and current status of sedative-hypnoticdrug use and abuse. Acta Psychiatr Scand 1986;73:465–78.Andersson PG. Ergotamine headache. Headache 1975;15:118–21.Baar HA. Treatment for headache: a four-step standardizedwithdrawal program for analgesic abusers. Pain Clin 1990;3:173–7 (Abstract).Bennett WM, DeBroe ME. Analgesic nephropathy: a preventablerenal disease. N Eng J Med 1989; 320:1269–71.Bowdler I, Killian J, Gänsslen-Blumberg S. The associationbetween analgesic abuse and headache – coincidental orcausal. Headache 1988; 28:494.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 99Braithwaite RA. The toxicity of tricyclic and newer antidepressants.In Handbook of Clinical Neurology (DeWolff FA,ed.). New York: Elsevier Science, 1995, pp. 311–20.Brust JC. Opiate addiction and toxicity. Ch. 16 in Handbook ofclinical neurology, vol. 65 (DeWolff FA, ed.). New York: ElsevierScience, 1995, pp. 356–61.Catarci T, Fiacco F, Argentino C. Ergotamine-inducedheadache can be sustained by sumatriptan daily intake.Cephalalgia 1994; 14:374–5.Centonze V, Polite BM, diBari M, Caporaletti P, Albano O. Vascularinjuries in ergotamine abuse: a case report. FunctNeurol 1993; 8:265–70.Dalquen P, Fasel J, Mihatsch MJ, Rist M, Rutishauser G.Phenacetinabusus IV. Sind zytologische harnuntersuchungenin der tumorvorsorge bei phenacetinabusern erfolgversprechendund anwendbar. Schweizerische MedizinischeWochenschrift 1980; 110:302–6.DeBroe ME, Elseviers MM. Analgesic nephropathy – still aproblem? Nephron 1993; 64:505–13.deMarinis M, Janiri L, Agnoli A. Headache in the use andwithdrawal of opiates and other associated substances ofabuse. Headache 1991; 31:159–63.Diamond S, Dalessio DJ. Drug abuse in headache. In Thepracticing physician’s approach to headache (Diamond S,Dalessio DJ, eds.). Williams & Wilkins, Baltimore. 1982, pp.114–21.Dichgans J, Diener HC. Clinical manifestations of excessiveuse of analgesic medication. In Drug – induced headache(Diener HC, Wilkinson M, eds.). Springer-Verlag, Berlin.1988, pp. 8–15.Dichgans J, Diener HD, Gerber WD et al. Analgetikainduzierterdauerkopfschmerz. Dtsch Med Wschr 1984,109:369–73.Diener HC. A personal view of the classification and definitionof drug dependence headache. Cephalalgia 1993;13:68–71.Diener HC, Dahlof CG. Headache associated with chronic useof substances. In The headaches (Olesen J, Tfelt-Hansen P,Welch KMA, eds.). Lippincott, Williams & Wilkins,Philadelphia. 1999, pp. 871–8.Diener HC, Dichgans J, Scholz E, Geiselhart S, Gerber WD,Bille A. Analgesic-induced chronic headache: long-termresults of withdrawal therapy. J Neurol 1989; 236:9–14.Diener HC, Haab J, Peters C, Ried S, Dichgans J, Pilgrim A.Subcutaneous sumatriptan in the treatment of headacheduring withdrawal from drug-induced headache.Headache 1991; 31:205–9.Diener HC, Pfaffenrath V, Soyka D, Gerber WD. Therapie desmedikamenten-induzierten dauerkopfschmerzes. MünchMed Wschr 1992; 134:159–62.Diener HC, Tfelt-Hansen P. Headache associated with chronicuse of substances. In The headaches (Olesen J, Tfelt-HansenP, Welch KMA, eds.). Raven press LTD, New York. 1993, pp.721–7.Dige-Petersen H, Lassen NA, Noer J, Toennesen KH, OlesenJ. Subclinical ergotism. Lancet 1977; i:65–6.Drucker P, Tepper S. Daily sumatriptan for detoxification fromrebound. Headache 1998; 38:687–90.Dubach UC, Rosner B, Pfister E. Epidemiologic study of abuseof analgesics containing phenacetin. Renal morbidity andmortality (1968–1979). N Engl J Med 1983; 308:357–62.Elkind AH. Drug abuse in headache patients. Clin J Pain 1989;5:111–20.Elkind AH. Drug abuse and headache. Med Clin N Am 1991;75:717–32.Evers S, Gralow I, Bauer B, Suhr B, Buchheister A, HusstedtIW, Ringelstein EB. Sumatriptan and ergotamine overuseand drug-induced headache: a clinicoepidemiologic study.Clin Neuropharmacol 1999; 22:201–6.Fanciullaci M, Alessandri M, Pietrini U, Briccolani-Bandini E,Beatrice S. Long-term ergotamine abuse: effect on adrenergicallyinduced mydriasis. Clin Pharm Ther 1992; 51:302–7.Fincham JE. Over-the-counter drug use and misuse by theambulatory elderly: a review of the literature. J Ger DrugTher 1987; 1:3–21.Fincham RW, Perdue Z, Dunn VD. Bilateral focal corticalatrophy and chronic ergotamine abuse. Neurology 1985;35:720–2.Fisher CM. Analgesic rebound headache refuted. Headache1988; 28:666.Friedman AP, Brazil P, vonStorch TJ. Ergotamine tolerance inpatients with migraine. JAMA 1955; 157:881–4.Gaist D, Hallas J, Sindrup SH, Gram LF. Is overuse of sumatriptana problem? A population-based study. Eur J ClinPharmacol 1996; 50:161–5.Gaist D, Tsiropoulus I, Sindrup SH, Hallas J, Rasmussen BK,Kragstrup J. Inappropriate use of sumatriptan: populationbased register and interview study. Br J Med 1998;316:1352–3.Granella F, Farina S, Malferrari G, Manzoni GC. Drug abusein chronic headache: a clinicoepidemiologic study. Cephalalgia1987; 7:15–9.Gutzwiller F, Zemp E. Der analgetikakonsum in derbevölkerung und socioökonomische aspekte des analgetikaabusus.In Das analgetikasyndrom (Mihatsch MJ, ed.).Thieme, Stuttgart. 1986, pp. 197–205.Hering R, Steiner TJ. Abrupt outpatient withdrawal frommedication in analgesic-abusing migraineurs. Lancet 1991;337:1442–3.Hokkanen E, Waltimo O, Kallanranta T. Toxic effects of ergotamineused for migraine. Headache 1978; 18:95–8.Horowski R, Ziegler A. Possible pharmacological mechanismsof chronic abuse of analgesics and other antimigrainedrugs. In Drug-induced headache (Diener HC, Wilkinson M,eds.). Springer-Verlag, Berlin. 1988, pp. 95–104.Horton BT, Peters GA. Clinical manifestations of excessive useof ergotamine preparations and management of withdrawaleffect: report of 52 cases. Headache 1963; 3:214–26.Isler H. Migraine treatment as a cause of chronic migraine. InAdvances in migraine research and therapy (Rose FC, ed.).Raven Press, New York. 1982, pp. 159–64.Jaffe JH. Drug addiction and drug abuse. In The pharmacologicalbasis of therapeutics (Gilman AG, Rall TW, Nies AS,Taylor P, eds.). Pergamon Press, New York. 1985, pp. 522–73.Katsarava Z, Fritsche G, Muessig M, Diener HC, Limmroth V.Clinical features of withdrawal headache following overuseof triptans and other headache drugs. Neurology 2001;57:1694–8.Kaube H, May A, Diener HC, Pfaffenrath V. Sumatriptanmisuse in daily chronic headache. Br Med J 1994; 308:1573.Kielholz P, Ladewig D. Probleme des medikamentenmi betabrauches.Schweiz Arztezeitung 1981; 62:2866–9.Diagnosing headache type (IHS 2003)100 ICHD-IIKlapper JA. Rebound headache: definition, symptomatology,treatment, and prevention. Headache Quarterly 1992;3:398–402.Kouyanou K, Pither CE, Rabe-Hesketh S, Wessely S. Acomparative study of iatrogenesis, medication abuse, andpsychiatric morbidity in chronic pain patients with andwithout medically explained symptoms. Pain 1998;76:417–26.Kudrow L. Paradoxical effects of frequent analgesic use. AdvNeurol 1982; 33:335–41.Lader M. Hypnotics and sedatives. In Handbook of clinical neurology(DeWolff FA, ed.). New York: Elsevier Science, 1995,pp. 329–55.Lance F, Parkes C, Wilkinson M. Does analgesic abuse causeheadache de novo? Headache 1988; 28:61–2.Lance JW. A concept of migraine and the search for the idealheadache drug. Headache 1990; 30:17–23.Limmroth V, Kazarawa S, Fritsche G, Diener HC. Headacheafter frequent use of new 5-HT agonists zolmitriptan andnaratriptan. Lancet 1999; 353:378.Limmroth V, Katsarava Z, Fritsche G, Przywara S, Diener HC.Features of medication overuse headache followingoveruse of different acute headache drugs. Neurology 2002;59:1011–14.Lucas RN, Falkowski W. Ergotamine and methysergide abusein patients with migraine. Br J Psychiatry 1973; 122:199–203.Ludolph AC, Husstedt IW, Schlake HP, Grotemeyer KH,Brune GG. Chronic ergotamine abuse: evidence of functionalimpairment of long ascending spinal tracts. EurNeurol 1988; 28:311–6.MacGregor EA, Vorah C, Wilkinson M. Analgesic use: a studyof treatments used by patients for migraine prior to attendingthe City of London migraine clinic. Headache 1990;30:634–8.Manzoni GC, Micieli G, Granella F, Sandrini G, Zanferrari C,Nappi G. Therapeutic approach to drug abuse in headachepatients. In Drug-induced headache (Diener HC, WilkinsonM, eds.). Springer-Verlag, Berlin. 1988, pp. 143–9.Marks V. Reactive (rebound) hypoglycemia. In Hypoglycemia(Marks V, Rose CF, eds.). Blackwell, Oxford. 1981, pp.179–217.Mathew NT. Amelioration of ergotamine withdrawal symptomswith naproxen. Headache 1987; 27:130–3.Mathew NT, Kurman R, Perez F. Drug induced refractoryheadache – clinical features and management. Headache1990; 30:634–8.Michultka DM, Blanchard EB, Appelbaum KA, Jaccard J,Dentinger MP. The refractory headache patient-2. Highmedication consumption (analgesic rebound) headache.Behav Res Ther 1989; 27:411–20.Micieli G, Manzoni GC, Granella F, Martignoni E, MalferrariG, Nappi G. Clinical and epidemiological observations ondrug abuse in headache patients. In Drug-Induced Headache(Diener HC, Wilkinson M, eds.). Springer-Verlag, Berlin.1988, pp. 20–8.Nicolodi M, DelBianco PL, Sicuteri F. The way to serotonergicuse and abuse in migraine. Int J Clin Pharmacol Res1997; 17:79–84.Page H. Rebound headache from ergotamine withdrawal.JAMA 1981; 246:719.Peters G, Horton BT. Headache: with special reference to theexcessive use of ergotamine preparations and withdrawaleffects. Proc Mayo Clin 1951; 26:153–61.Pini LA, Trenti T. Case report: does chronic use of sumatriptaninduce dependence? Headache 1994; 34:600–1.Pradalier A, Dry S, Baron JF. Cephalée induite par l’abuse detartrate d’ergotamine chez les migrainieux. Concours Méd1984; 106:106–10.Rahman A, Segasothy M, Samad SA, Zulfiqar A, Rani M.Analgesic use and chronic renal disease in patients withheadache. Headache 1993; 33:442–5.Rapoport A, Stang P, Gutterman DL, Cady R, Markley H,Weeks R, Saiers J, Fox AW. Analgesic rebound headache inclinical practice: data from a physician survey. Headache1996; 36:14–9.Rapoport AM. Analgesic rebound headache. Headache 1988;28:662–5.Rapoport AM, Weeks RE. Characteristics and treatment ofanalgesic rebound headache. In Drug-induced headache(Diener HC, Wilkinson M, eds.). Springer-Verlag, Berlin.1988, pp. 162–7.Roswell AR, Neylan C, Wilkinson M. Ergotamine inducedheadache in migrainous patients. Headache 1973; 13:65–7.Sandler DP, Smith JC, Weinberg CR, Buckalew VM, DennisVW, Blythe WB, Burgess WP. Analgesic use and chronicrenal disease. N Engl J Med 1989; 320:1238–43.Saper JR. Drug abuse among headache patients. In Headachedisorders (Saper JR, ed.). PSG Publishers, Boston. 1983, pp.263–78.Saper JR. Drug overuse among patients with headache.Neurol Clin 1983; 1:465–77.Saper JR. Daily chronic headaches. Neurol Clin N Amer 1990;8:891–902.Saper JR, Jones JM. Ergotamine tartrate dependency: featuresand possible mechanisms. Clin Neuropharmacol 1986;9:244–56.Schnider P, Aull S, Baumgartner C et al. Long-term outcomeof patients with headache and drug abuse after inpatientwithdrawal: five-year followup. Cephalalgia 1996; 16:481–5.Schnider P, Aull S, Feucht M. Use and abuse of analgesics intension-type headache. Cephalalgia 1994; 14:162–7.Schnider P, Maly J, Grunberger J, Aull S, Zeiler K, Wessely P.Improvement of decreased critical flicker frequency inheadache patients with drug abuse after successful withdrawal.Headache 1995; 35:269–72.Schoenen J, Lenarduzzi P, Sianard-Gainko J. Chronicheadaches associated with analgesics and/or ergotamineabuse: a clinical survey of 434 consecutive outpatients. InNew advances in headache research (Rose FD, ed.). Smith-Gordon, London. 1989, pp. 29–43.Seller EM, Busto UE, Kaplan HL, Somer G, Baylon GJ. Comparativeabuse liability of codeine and naratriptan. ClinPharmacol Ther 1998; 63:121.Shakir RA. Vitamin toxicity. In Handbook of clinical neurology,vol. 65 (DeWolff FA, ed.). New York: Elsevier Science, 1995,pp. 567–76.Silberstein SD, Lipton RB. Chronic daily headache. InHeadache (Goadsby PJ, Silberstein SD, eds.). Butterworth-Heinemann, Newton. 1997, pp. 201–25.Silberstein SD, Lipton RB, Solomon S, Mathew NT. Classificationof daily and near daily headaches: proposed revisionsto the IHS classification. Headache 1994; 34:1–7.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 101Silberstein SD, Silberstein JR. Chronic daily headache: prognosisfollowing inpatient treatment with repetitive IV DHE.Headache 1992; 32:439–45.Stewart JH. Analgesic abuse and renal failure in Australia.Kidney International 1978; 13:72–8.Sullivan JT, Sellers EM. Treatment of the barbiturate abstinencesyndrome. Med J Aust 1986; 145:456–8.Taschner KL, Wiesbeck GA. Psychiatric aspects of drug addictionof the barbiturate-alcohol type. In Drug-inducedheadache (Diener HC, Wilkinson M, eds.). Berlin, Springer-Verlag. 1988, pp. 80–4.Tfelt-Hansen P. Ergotamine headache. In Updating in headache(Pfaffenrath V, Lundberg P, Sjaastad O, eds.). Springer,Berlin. 1985, pp. 169–72.Tfelt-Hansen P. The effect of ergotamine on the arterial systemin man. Acta Pharmacol Toxicol 1986; 59:1–29.Tfelt-Hansen P, Krabbe AA. Ergotamine. Do patients benefitfrom withdrawal? Cephalalgia 1981; 1:29–32.Tfelt-Hansen P, Olesen J. Arterial response to ergotamine tartratein abusing and non-abusing migraine patients. ActaPhysiol Scand 1981; 48:69–72.Tfelt-Hansen P, Paalzow L. Intramuscular ergotamine: plasmalevels and dynamic activity. Clin Pharmacol Ther 1985;37:29–35.Tfelt-Hansen P, Saxena PR, Ferrari MD. Ergot alkaloids. InHandbook of clinical neurology (DeWolff FA, ed.). New York:Elsevier Science. 1995, pp. 61–7.Timmings PL, Richens A. Neurotoxicology of antiepilepticdrugs. In Handbook of clinical neurology (DeWolff FA, ed.).New York: Elsevier Science. 1995, pp. 495–513.Vasconcellos E, Pina-Garza JE, Millan EJ, Warner JS. Analgesicrebound headache in children and adolescents. J ChildNeurol 1998; 13:443–7.Verhoeff NP, Visser WH, Ferrari MD, Saxena PR, vonRoyenEA. Dopamine D2 receptor imaging with 123-I-iodobenzamideSPECT in migraine patients abusing ergotamine: doesergotamine cross the blood brain barrier. Cephalalgia 1993;13:325–9.VonKorff M, Galer BS, Stang P. Chronic use of symptomaticheadache medications. Pain 1995; 62:179–86.Walker J, Parisi S, Olive D. Analgesic rebound headache:experience in a community hospital. Southern Med J 1993;86:1202–5.Warner JS. Rebound headaches: a review. Headache Quarterly1999; 10:207–19.Zed PJ, Loewen PS, Robinson G. Medication-inducedheadache: overview and systematic review of therapeuticapproaches. Ann Pharmacother 1999; 33:61–72.Ziegler DK. Opiate and opioid use in patients with refractoryheadache. Cephalalgia 2000; 14:5–10.8.3 Headache as an adverse event attributed tochronic medicationDalton K. Migraine and oral contraceptives. Headache 1976;15: 247–51.de Lignières B, Silberstein SD. Pharmacodynamics of oestrogensand progestagens. Cephalalgia 2000; 20:200–7.Magos AL, Brewster E, Singh R, O’Dowd T, Brincat M, StuddJWW. The effects of norethisterone in postmenopausalwomen on oestrogen replacement therapy: a model for thepremenstrual syndrome. Br J Obstet Gynaecol 1986;93:1290–6.Nappi RE, Cagnacci A, Granella F, Piccinini F, Polatti F,Facchinetti F. Course of primary headaches duringhormone replacement therapy. Maturitas 2001; 38:157–63.Silberstein SD. Hormone-related headache. M Clin N Am2001; 85:1017–35.Silberstein SD, de Lignières B. Migraine, menopause and hormonalreplacement therapy. Cephalalgia 2000; 20:214–2.8.4 Headache attributed to substance withdrawalAbbott PJ. Caffeine: a toxicological overview. Med J Aust 1986;145:518–21.Baumgartner GR, Rowen RC. Transdermal clonidine versuschlordiazepoxide in alcohol withdrawal: a randomized,controlled clinical trial. South Med J 1991; 84:312–21.Dalessio DJ. (1980). Wolff’s headache and other head pain, OxfordUniversity Press, Oxford.Epstein MT, Hockaday JM, Hockaday TDR. Migraine andreproductive hormones through the menstrual cycle. Lancet1975; i:543–8.Greden JF, Fontaine M, Lubetsky M, Chamberlin K. Anxietyand depression associated with caffeinism among psychiatricinpatients. Am J Psychiatr 1978; 135:963–6.Laska EM, Sunshine A, Mueller F, Elvers WB, Siegel C, RubinA. Caffeine as an analgesic adjuvant. JAMA 1984;251:1711–8.Lichten E, Lichten J, Whitty A, Pieper D. The confirmation ofa biochemical marker for women’s hormonal migraine: thedepo-oestradiol challenge test. Headache 1996; 36: 367–71.Raskin NH, Appenzeller O. Headache, Saunders, Philadelphia.1980.Silverman K, Evans SM, Strain EC, Griffiths RR. Withdrawalsyndrome after the double-blind cessation of caffeine consumption.N Eng J Med 1992; 327:1109–14.Somerville B. Estrogen withdrawal migraine. Neurology 1975;25:239–50.vanDusseldorp M, Katan MB. Headache caused by caffeinewithdrawal among moderate coffee drinkers switched fromordinary to decaffeinated coffee: a 12 week double blindtrial. Br Med J 1990; 300:1558–9.White BC, Lincoln CA, Pearcz NW, Reeb R, Vaida C. Anxietyand muscle tension as consequence of caffeine withdrawal.Science 1980; 209:1547–8.Diagnosing headache type (IHS 2003)102 ICHD-II9. Headache attributed to infection9.1 Headache attributed to intracranial infection9.1.1 Headache attributed to bacterialmeningitis9.1.2 Headache attributed to lymphocyticmeningitis9.1.3 Headache attributed to encephalitis9.1.4 Headache attributed to brain abscess9.1.5 Headache attributed to subduralempyema9.2 Headache attributed to systemic infection9.2.1 Headache attributed to systemic bacterialinfection9.2.2 Headache attributed to systemic viralinfection9.2.3 Headache attributed to other systemicinfection9.3 Headache attributed to HIV/AIDS9.4 Chronic post-infection headache9.4.1 Chronic post-bacterial meningitisheadacheCoded elsewhere:Headache disorders attributed to extracranial infectionsof the head (such as ear, eye and sinus infections)are coded as subtypes of 11. Headache or facialpain attributed to disorder of cranium, neck, eyes, ears,nose, sinuses, teeth, mouth or other facial or cranialstructures.General commentPrimary or secondary headache or both?When a new headache occurs for the first time inclose temporal relation to an infection, it is coded asa secondary headache attributed to the infection.This is also true if the headache has the characteristicsof migraine, tension-type headache or clusterheadache. When a pre-existing primary headache ismade worse in close temporal relation to an infection,there are two possibilities, and judgment isrequired. The patient can either be given only thediagnosis of the pre-existing primary headache or begiven both this diagnosis and the diagnosis ofheadache attributed to the infection. Factors thatsupport adding the latter diagnosis are: a very closetemporal relation to the infection, a marked worseningof the pre-existing headache, very good evidencethat the infection can aggravate the primaryheadache and, finally, improvement or resolution ofthe headache after relief from the infection.Definite, probable or chronic?A diagnosis of Headache attributed to an infectionusually becomes definite only when the headacheresolves or greatly improves after effective treatmentor spontaneous remission of the infection. If theinfection cannot be treated effectively or does notremit spontaneously, or when there has been insufficienttime for this to happen, a diagnosis of Headacheprobably attributed to infection is usually applied.This is not the case for 9.1.1 Headache attributed tobacterial meningitis. It is recognised that thisheadache may become chronic. When the causativeinfection is effectively treated or remits spontaneouslybut headache persists after 3 months, thediagnosis changes to 9.4.1 Chronic post-bacterialmeningitis headache.In other cases when the infection is eliminated butheadache does not resolve or markedly improveafter 3 months, a diagnosis of A9.4.2 Chronic postnon-bacterial infection headache may be considered.This is described only in the appendix as suchheadaches have been poorly documented, andresearch is needed to establish better criteria forcausation.IntroductionHeadache is a common accompaniment of systemicviral infections such as influenza. It is also commonwith sepsis; more rarely it may accompany other systemicinfections.In intracranial infections headache is usually thefirst and the most frequently encountered symptom.Occurrence of a new type of headache which isdiffuse, pulsating and associated with a generalfeeling of illness and/or fever should direct attentiontowards an intracranial infection even in the absenceof a stiff neck. Unfortunately, there are no goodprospective studies of the headaches associated withintracranial infection and precise diagnostic criteriafor these subtypes of headache cannot be developedin all cases.9.1 Headache attributed to intracranial infection9.1.1 Headache attributed to bacterial meningitisDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. diffuse pain2. intensity increasing to severe3. associated with nausea, photophobia and/orphonophobiaDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 103B. Evidence of bacterial meningitis from examinationof CSFC. Headache develops during the meningitisD. One or other of the following:1. headache resolves within 3 months after relieffrom meningitis2. headache persists but 3 months have not yetpassed since relief from meningitisDiagnosing headache type comments:Headache is the commonest and may be the firstsymptom of bacterial meningitis. Headache is a keysymptom of meningeal syndrome or meningismconsisting usually of headache, neck stiffness andphotophobia.A variety of microorganisms may cause primaryor secondary meningitis. Direct stimulation of thesensory terminals located in the meninges by bacterialinfection causes the onset of headache. Bacterialproducts (toxins), mediators of inflammation such asbradykinin, prostaglandins and cytokines and otheragents released by inflammation not only directlycause pain but also induce pain sensitisation andneuropeptide release.When headache persists after 3 months, code as9.4.1 Chronic post-bacterial meningitis headache.9.1.2 Headache attributed to lymphocyticmeningitisDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. acute onset2. severe intensity3. associated with nuchal rigidity, fever, nausea,photophobia and/or phonophobiaB. Examination of CSF shows lymphocytic pleocytosis,mildly elevated protein and normal glucose1C. Headache develops in close temporal relation tomeningitisD. Headache resolves within 3 months2 after successfultreatment or spontaneous remission ofinfectionNotes:1. Virus, borrelia, listeria, fungus, tuberculosis orother infective agent(s) may be identified byappropriate methods.2. Headache usually resolves within 1 week.Diagnosing headache type comments:Headache, fever, photophobia and nuchal rigidityare the main symptoms of lymphocytic or nonbacterialmeningitis and headache may remain as themain symptom throughout the course of the disease.Headache can appear with intracranial infectionbut also in systemic inflammation. Since the signs ofsystemic inflammation associated with headachedo not necessarily mean meningitis or encephalitis,diagnosis of lymphocytic meningitis must be confirmedby CSF examination.Enteroviruses account for most viral causes.Herpes simplex, adenovirus, mumps and othersmay also be responsible.9.1.3 Headache attributed to encephalitisDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. diffuse pain2. intensity increasing to severe3. associated with nausea, photophobia orphonophobiaB. Neurological symptoms and signs of acuteencephalitis, and diagnosis confirmed by EEG,CSF examination, neuroimaging and/or otherlaboratory investigations1C. Headache develops during encephalitisD. Headache resolves within 3 months after successfultreatment or spontaneous remission of theinfectionNote:1. PCR method gives the specific diagnosis.Diagnosing headache type comments:The causes of headache include both meningeal irritationand increased intracranial pressure. Headpain may also be a systemic reaction to the toxicproducts of the infecting agent(s). Headache mayoccur early and be the only clinical symptom ofencephalitis.Herpes simplex virus, arbovirus and mumps areknown causes of encephalitis. Except for HSVencephalitis (in which 95% of cases are identifiablewith PCR), the causative virus is identified in fewerthan half of cases of encephalitis.9.1.4 Headache attributed to brain abscessDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. constant pain3. intensity gradually increasing to moderate orsevereDiagnosing headache type (IHS 2003)104 ICHD-II4. aggravated by straining5. accompanied by nauseaB. Neuroimaging and/or laboratory evidence ofbrain abscessC. Headache develops during active infectionD. Headache resolves within 3 months after successfultreatment of the abscessDiagnosing headache type comments:Direct compression and irritation of the meningealor arterial structures and increased intracranial pressureare the mechanisms for causing headache.The most common organisms causing brainabscess include streptococcus, staphylococcusaureus, bacteroides species and enterobacter. Predisposingfactors include infections of paranasalsinuses, ears, jaws, teeth or lungs.9.1.5 Headache attributed to subdural empyemaDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. unilateral or much more intense on one side2. associated with tenderness of the skull3. accompanied by fever4. accompanied by stiffness of the neckB. Neuroimaging and/or laboratory evidence ofsubdural empyemaC. Headache develops during active infection andis localised to or maximal at the site of theempyemaD. Headache resolves within 3 months after successfultreatment of the empyemaDiagnosing headache type comments:Headache is caused by meningeal irritation,increased intracranial pressure and/or fever.Subdural empyema is often secondary to sinusitisor otitis media. It may also be a complication ofmeningitis. Early diagnosis is best made by CT orMRI.9.2 Headache attributed to systemic infectionCoded elsewhere:Headache attributed to meningitis or encephalitisaccompanying systemic infection should be codedaccordingly under 9.1 Headache attributed to intracranialinfection.Diagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. diffuse pain2. intensity increasing to moderate or severe3. associated with fever, general malaise or othersymptoms of systemic infectionB. Evidence of systemic infectionC. Headache develops during the systemic infectionD. Headache resolves within 72 hours after effectivetreatment of the infectionDiagnosing headache type comments:Headache in systemic infections is usually a relativelyinconspicuous symptom and diagnosticallyunhelpful. These conditions are mostly dominatedby fever, general malaise and systemic symptoms.Nevertheless, some systemic infections, particularlyinfluenza, have headache as a prominent symptomalong with fever and other symptoms. In other cases,systemic infection is accompanied by meningitis orencephalitis, and the headache should be coded tothese disorders.The great variability in their propensity forcausing headache indicates that systemic infectionsdo not have this effect simply through fever. Themechanisms causing headache include direct effectsof the microorganisms themselves. In infectiousdisease, headache commonly coexists with fever andmay be dependent on it, but headache can occur inthe absence of fever. The presence or absence of fevermay be used in the differential classification ofheadache. The exact cause of headache by fever isnot elucidated. Some infective microorganisms mayinfluence brainstem nuclei which release substancesto cause headache, or endotoxins may activateinducible NOS causing production of nitric oxide(NO). The exact nature of these mechanisms remainsto be investigated.9.2.1 Headache attributed to systemic bacterialinfectionDiagnosing headache type diagnostic criteria:A. Headache fulfilling criteria for 9.2 Headache attributedto systemic infectionB. Laboratory investigation discloses the inflammatoryreaction and identifies the organismDiagnosing headache type comment:Some infective agents have a particular tropism forthe central nervous system. They may activate brainstemnuclei where release of toxins inducesheadache mechanisms.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 1059.2.2 Headache attributed to systemic viralinfectionDiagnosing headache type diagnostic criteria:A. Headache fulfilling criteria for 9.2 Headacheattributed to systemic infectionB. Clinical and laboratory (serology and/or PCRmolecular) diagnosis of viral infection9.2.3 Headache attributed to other systemicinfectionDiagnosing headache type diagnostic criteria:A. Headache fulfilling criteria for 9.2 Headache attributedto systemic infectionB. Clinical and laboratory (serology, microscopy,culture or PCR molecular) diagnosis of infectionother than bacterial or viral9.3 Headache attributed to HIV/AIDSCoded elsewhere:Headache attributed to a specific supervening infectionis coded according to that infection.Diagnosing headache type diagnostic criteria:A. Headache with variable mode of onset, site andintensity1 fulfilling criteria C and DB. Confirmation of HIV infection and/or of thediagnosis of AIDS, and of the presence ofHIV/AIDS-related pathophysiology likely tocause headache2, by neuroimaging, CSF examination,EEG and/or laboratory investigationsC. Headache develops in close temporal relation tothe HIV/AIDS-related pathophysiologyD. Headache resolves within 3 months after theinfection subsidesNotes:1. Headache as a symptom of HIV infection is dulland bilateral. Otherwise. the onset, site andintensity of headache vary according to theHIV/AIDS-related conditions (such as meningitis,encephalitis or systemic infection) that arepresent.2. see diagnosing headache type comments.Diagnosing headache type comments:Dull bilateral headache may be a part of the symptomatologyof HIV infection. Headache may also beattributed to aseptic meningitis during HIV infection(but not exclusively in the AIDS stages) and to secondarymeningitis or encephalitis associated withopportunistic infections or neoplasms (which mostlyoccur in the AIDS stages). The most commonintracranial infections in HIV/AIDS are toxoplasmosisand cryptococcal meningitis.Headache occurring in patients with HIV/AIDSbut attributed to a specific supervening infection iscoded to that infection.9.4 Chronic post-infection headache9.4.1 Chronic post-bacterial meningitis headacheDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. diffuse continuous pain2. associated with dizziness3. associated with difficulty in concentratingand/or loss of memoryB. Evidence of previous intracranial bacterial infectionfrom CSF examination or neuroimagingC. Headache is a direct continuation of 9.1.1Headache attributed to bacterial meningitisD. Headache persists for >3 months after resolutionof infectionDiagnosing headache type comments:A reported 32% of survivors of bacterial meningitissuffer from persistent headache (Bohr et al., 1983).There is no evidence for persistent headachefollowing other infections, but criteria for A9.4.2Chronic post-non-bacterial infection headache are in theappendix. More research is needed.Bibliography and reference9.1.1 Headache attributed to bacterial meningitisDrexler ED. Severe headache: when to worry, what to do.Postgrad Med 1990; 87:164–70, 173–80.Francke E. The many causes of meningitis. Postgrad Med1987; 82:175–8, 181–3, 187–8.Gedde-Dahl TW, Lettenstrom GS, Bovre K. Coverage formeningococcal disease in the Norwegian morbidity andmortality statistics. NIPH Ann 1980; 3(2):31–5.Jones HR, Siekert RG. Neurological manifestation of infectiveendocarditis. Brain 1989; 112:1295–315.Tonjum T. Nilsson F, Bruun JH, Hanebeg B. The early phaseof meningococcal disease. NIPH Ann 1983; 6:175–81.Zhang SR, Zhang YS, Zhao XD. Tuberculous meningitis withhydrocephalus: a clinical and CT study. Chung Hua Nei KoTsa Chih 1989; 28:202–4.9.1.2 Headache attributed to lymphocyticmeningitisCochius JI, Burns RJ, Willoughby JO. CNS cryptococcosis:unusual aspects. Clin Exp Neurol 1989; 26:183–91.Dalton M, Newton RW. Aseptic meningitis. Dev Med ChildNeurol 1991; 33:446–58.Diagnosing headache type (IHS 2003)106 ICHD-IIGomez-Arada F, Canadillas F, Marti-Masso FJ et al. Pseudomigrainewith temporary neurological symptoms and lymphocyticpleocytosis. Brain 1997; 120:1105–13.Mak SC, Jeng JE, Jong JY, Chiang CH, Chou LC. Clinical observationsand virological study of aseptic meningitis in theKaohsinug area. Taiwan I Hsueh Hui Twa Chih 1990;89:868–72.Pachner AR, Steere AC. Neurological findings of Lymedisease. Yale Biol Med 1984; 57:481–3.Pachner AR, Steere AC. The triad of neurologic manifestationsof Lyme disease: meningitis, cranial neuritis, and radiculoneuritis.Neurology 1985; 35:47–53.Singer JI, Maur PR, Riley JP, Smith PB. Management of centralnervous system infections during an epidemic of enteroviralaseptic meningitis. J Pediatr 1980; 96:559–63.9.1.3 Headache attributed to encephalitisBrooks RG, Licitra CM, Peacock MG. Encephalitis caused byCoxiella burnetii. Ann Neurol 1986; 20:91–3.Davis LE, McLaren LC. Relapsing herpes simplex encephalitisfollowing antiviral therapy. Ann Neurol 1983; 13:192–5.Domachowske JB, Cunningham CK, Cummings DL, CrosleyCJ, Hannan WP, Weiner LB. Acute manifestations and neurologicsequelae of Epstein-Barr virus encephalitis in children.Pediatr Infect Dis J 1996; 15:871–5.Kennedy PG. Retrospective analsys of 46 cases of simplexencephalitis seen in Glasgow between 1962 and 1985. OJM1988; 86:533–40.Kennedy PG, Adams IH, Graham DI, Clements GB. A clinicopathologicalstudy of herpes simplex encephalitis. NeuropatholAppl Neurobiol 1998; 14:395–415.Poneprasert B. Japanese encephalitis in children in northernThailand. Southeast Asian J Trop Med Public health 1989;20:599–603.Saged JI, Weinstein Mo, Miller DC. Chronic encephalitis possiblydue to herpes simplex virus: two cases. Neurology1985; 35:1470–2.9.1.4 Headache attributed to brain abscessChalstrey S, Pfleiderer AG, Moffat DA. Persisting incidenceand mortality of sinogenic cerebral abscess: a continuingreflection of late clinical diagnosis. J R Soc Med 1991;84:193–5.Chun CH, Johnson JD, Hofstetter M, Raff MJ. Brain abscess: astudy of 45 consecutive cases. Medicine 1986; 65:415–31.Harris LF, Maccubbin DA, Triplett JN, Haws FB. Brainabscess: recent experience at a community hospital. SouthMed J 1985; 78:704–7.Kulay A, Ozatik N, Topucu I. Otogenic intracranial abscesses.Acta Neurochir (Wien) 1990; 107:140–6.Yen PT, Chan ST, Huang TS. Brain abscess: with spcial referenceto otolaryngologic sources of infection. OtolaryngolHead Neck Surg 1995; 113:15–22.9.1.5 Headache attributed to subdural empyemaHodges J, Anslow P, Gillet G. Subdural empyema: continuingdiagnostic problems in the CT scan era. QJM 1986;59:387–93.McIntyre PB, Lavercombe PS, Kemp RJ, McCormack JG. Subduraland epidural empyema: diagnostic and therapeuticproblems. Med J Aust 1991; 154:653–7.Sellik JA. Epidural abscess and subdural empyema. J AmOsteopath Assoc 1989; 89:806–10.9.2 Headache attributed to systemic infectionDe Marinis M, Welch KM, Headache associated with noncephalicinfections: classification and mechanisms. Cephalalgia1992; 12:197–201.9.3 Headache attributed to HIV/AIDSBrew BJ, Miller J. Human immunodeficiency virus-relatedheadache. Neurology 1993; 43:1098–100.Denning DW. The neurological features of HIV infection.Biomed Pharmacother 1988; 42:11–4.Evers S, Wibbeke B, Reichelt D, Suhr B, Brilla R, Husstedt IW.The impact of HIV infection on primary headache. Unexpectedfindings from retrospective, cross-sectional, andprospective analyses. Pain 2000; 85:191–200.Hollander H, Strimgari S. Human immunodeficiency virusassociatedmeningitis. Clinical course and correlations. AmJ Med 1987; 83:813–6.Rinaldi R, Manfredi R, Azzimondi G et al. Recurrent‘migrainelike’ episodes in patients with HIV disease.Headache 1007; 37:443–8.Weinke T, Rogler G, Sixt C et al. Cryptococcosis in AIDSpatients: observations concerning CNS involvement. JNeurol 1989; 236:38–42.9.4 Chronic post infection headacheBohr V, Hansen B, Kjersen H, Rasmussen N, Johnsen N, KristensenHS, Jessen O. Sequelae from bacterial meningitis andtheir relation to the clinical condition during acute illness,based on 667 questionnaire returns. Part II of a three partseries. J Infect 1983; 7:102–10.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 10710. Headache attributed to disorder ofhomoeostasis10.1 Headache attributed to hypoxia and/orhypercapnia10.1.1 High-altitude headache10.1.2 Diving headache10.1.3 Sleep apnoea headache10.2 Dialysis headache10.3 Headache attributed to arterial hypertension10.3.1 Headache attributed tophaeochromocytoma10.3.2 Headache attributed to hypertensivecrisis without hypertensiveencephalopathy10.3.3 Headache attributed to hypertensiveencephalopathy10.3.4 Headache attributed to pre-eclampsia10.3.5 Headache attributed to eclampsia10.3.6 Headache attributed to acute pressorresponse to an exogenous agent10.4 Headache attributed to hypothyroidism10.5 Headache attributed to fasting10.6 Cardiac cephalalgia10.7 Headache attributed to other disorder ofhomoeostasisCoded elsewhere:7.1.2 Headache attributed to intracranial hypertensionsecondary to metabolic, toxic or hormonal causes.General commentPrimary or secondary headache or both?When a new headache occurs for the first time inclose temporal relation to a disorder of homoeostasis,it is coded as a secondary headache attributed tothat disorder. This is also true if the headache has thecharacteristics of migraine, tension-type headacheor cluster headache. When a pre-existing primaryheadache is made worse in close temporal relationto a disorder of homoeostasis, there are two possibilities,and judgment is required. The patient caneither be given only the diagnosis of the pre-existingprimary headache or be given both this diagnosisand the diagnosis of headache attributed to the disorderof homoeostasis. Factors that support addingthe latter diagnosis are: a very close temporal relationto the disorder of homoeostasis, a marked worseningof the pre-existing headache, very goodevidence that the disorder of homoeostasis canaggravate the primary headache and, finally,improvement or resolution of the headache afterrelief from the disorder of homoeostasis.Definite, probable or chronic?A diagnosis of Headache attributed to disorder ofhomoeostasis usually becomes definite only when theheadache resolves or greatly improves after effectivetreatment or spontaneous remission of the disorder.If this disorder cannot be treated effectively or doesnot remit spontaneously, or when there has beeninsufficient time for this to happen, a diagnosis ofHeadache probably attributed to disorder of homoeostasisis usually applied.The alternative, when the disorder of homoeostasisis effectively treated or remits spontaneously butheadache does not resolve or markedly improveafter 3 months, is a diagnosis of A10.8 Chronic posthomoeostasisdisorder headache. This is described onlyin the appendix as such headaches have been poorlydocumented, and research is needed to establishbetter criteria for causation.IntroductionHeadache disorders described here were previouslyreferred to as Headache associated with metabolic or systemicdisease. However, Headache attributed to disorderof homoeostasis was felt to capture more accuratelythe true nature of these headache disorders.Headaches caused by significant disturbances inarterial pressure and by myocardial ischaemia arenow included in this section. In addition, disordersof homoeostatic mechanisms affecting a variety oforgan systems, including altered arterial bloodgases, volume disturbances as in dialysis and disordersof endocrine function, are covered here.Headache attributed to fasting is also included.10.1 Headache attributed to hypoxia and/or hypercapniaDiagnosing headache type comments:Headache occurs within 24 hours after acute onsetof hypoxia with PaO2 <70mmHg or in chronicallyhypoxic patients with PaO2 persistently at or belowthis level.It is often difficult to separate the effects ofhypoxia and hypercapnia.10.1.1 High-altitude headacheDiagnosing headache type diagnostic criteria:A. Headache with at least two of the following characteristicsand fulfilling criteria C and D:1. bilateral2. frontal or frontotemporalDiagnosing headache type (IHS 2003)108 ICHD-II3. dull or pressing quality4. mild or moderate intensity5. aggravated by exertion, movement, straining,coughing or bendingB. Ascent to altitude above 2500mC. Headache develops within 24 hours after ascentD. Headache resolves within 8 hours after descentDiagnosing headache type comments:Headache is a frequent complication of ascent to altitude– occurring in more than 80% of cases. 10.1.1High-altitude headache appears to be independentof an individual’s previous history of headache,although patients with migraine may describe moresevere headache that resembles their typicalmigraine attacks.Acute mountain sickness (AMS) consists of at leastmoderate headache combined with one or more ofnausea, anorexia, fatigue, dizziness and sleep disturbances.Acetazolamide (125mg, two or threetimes daily) may reduce susceptibility to acutemountain sickness. Preventative strategies includeallowing two days of acclimatisation prior to engagingin strenuous exercise at high altitudes, avoidingalcohol and liberalising fluid intake. Most highaltitudeheadaches respond to simple analgesicssuch as paracetamol (acetaminophen) or ibuprofen.10.1.2 Diving headacheCoded elsewhere:1. Migraine, 2. Tension-type headache, 4.3 Primary exertionalheadache, 11.2.1 Cervicogenic headache, 13.6Supraorbital neuralgia, 13.10 External compressionheadache and 13.11 Cold-stimulus headache precipitatedby diving are coded as those disorders.Diagnosing headache type diagnostic criteria:A. Headache, no typical characteristics known, fulfillingcriteria C and DB. Diving to depth below 10mC. Headache develops during diving and is accompaniedby at least one of the following symptomsof CO2 intoxication in the absence of decompressionillness:1. light-headedness2. mental confusion3. dyspnoea4. flushed feeling in the face5. motor incoordinationD. Headache resolves within 1 hour after treatmentwith 100% O2Diagnosing headache type comments:Hypercapnia (arterial PCO2 >50mmHg) is known tocause relaxation of cerebrovascular smooth muscleand lead to vasodilatation and increased intracranialpressure. There is some evidence that hypercapniain the absence of hypoxia is associated withheadache. The best clinical example of headacheattributed to hypercapnia occurs in divers. Carbondioxide may accumulate in a diver who intentionallyholds his or her breath intermittently (skipbreathing) in a mistaken attempt to conserve air, ortakes shallow breaths to minimise buoyancy variationsin the narrow passages of a wreck or cave.Divers may also hypoventilate unintentionally whena tight wetsuit or buoyancy compensator jacketrestricts chest wall expansion, or when ventilation isinadequate in response to physical exertion. Strenuousexercise increases the rate of CO2 productionmore than 10-fold, resulting in a transient elevationof PCO2 to more than 60mmHg. Diving headacheusually intensifies during the decompression phaseof the dive or upon resurfacing.Mild non-specific headache is also common indivers with decompression illness, and may be associatedwith musculoskeletal pain and, in moreserious cases, with focal neurological and/or respiratorysymptoms, loss of consciousness and/or cognitivedeficits.Headache in divers can also occur as a result ofcarbon monoxide intoxication which rarely contaminatesdivers’ compressed-air supply if the air intakesystem is positioned in such a way as to gatherimproperly directed combustion-engine exhaust.Such headache is coded as 8.1.3 Carbon monoxideinducedheadache.Migraine, tension-type headache, primary exertionalheadache, cervicogenic headache, supraorbitalneuralgia, external compression headache andcold-stimulus headache can occur during a dive, butdiving in these instances should be considered a precipitatingfactor rather than the cause.10.1.3 Sleep apnoea headacheDiagnosing headache type diagnostic criteria:A. Recurrent headache with at least one of the followingcharacteristics and fulfilling criteria C andD:1. occurs on >15 days per month2. bilateral, pressing quality and not accompaniedby nausea, photophobia or phonophobia3. each headache resolves within 30 minutesB. Sleep apnoea (Respiratory Disturbance Index ≥5)demonstrated by overnight polysomnographyDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 109C. Headache is present upon awakeningD. Headache ceases within 72 hours, and does notrecur, after effective treatment of sleep apnoeaDiagnosing headache type comments:Although morning headache is significantly morecommon in patients with sleep apnoea than in thegeneral population, headache present upon awakeningis a non-specific symptom which occurs in avariety of primary and secondary headache disorders,in sleep-related respiratory disorders otherthan sleep apnoea (eg, Pickwickian syndrome,chronic obstructive pulmonary disorder), and inother primary sleep disorders such as periodic legmovements of sleep. A definitive diagnosis of 10.1.3Sleep apnoea headache requires overnightpolysomnography.It is unclear whether the mechanism of 10.1.3 Sleepapnoea headache is related to hypoxia, hypercapnia ordisturbance in sleep.10.2 Dialysis headacheDiagnosing headache type diagnostic criteria:A. At least 3 attacks of acute headache fulfilling criteriaC and DB. The patient is on haemodialysisC. Headache develops during at least half ofhaemodialysis sessionsD. Headache resolves within 72 hours after eachhaemodialysis session and/or ceases altogetherafter successful transplantationDiagnosing headache type comments:Headache commonly occurs in association withhypotension and dialysis disequilibrium syndrome.The disequilibrium syndrome may begin asheadache and then progress to obtundation andfinally coma, with or without seizures. This syndromeis relatively rare and may be prevented bychanging dialysis parameters.As caffeine is rapidly removed by dialysis, 8.4.1Caffeine-withdrawal headache should be considered inpatients who consume large quantities of caffeine.10.3 Headache attributed to arterial hypertensionDiagnosing headache type comment:Mild (140–159/90–99mmHg) or moderate (160–179/100–109mmHg) chronic arterial hypertensiondoes not appear to cause headache. Whether moderatehypertension predisposes to headache at allremains controversial, but there is little evidence thatit does. Ambulatory blood pressure monitoring inpatients with mild and moderate hypertension hasshown no convincing relationship between bloodpressure fluctuations over a 24-hour period andpresence or absence of headache.10.3.1 Headache attributed to phaeochromocytomaDiagnosing headache type diagnostic criteria:A. Intermittent discrete attacks of headache accompaniedby at least one of the following and fulfillingcriteria C and D:1. sweating2. palpitations3. anxiety4. pallorB. Phaeochromocytoma demonstrated by biochemicalinvestigations, imaging and/or surgeryC. Headache develops concomitantly with abruptrise in blood pressureD. Headache resolves or markedly improves within1 hour of normalisation of blood pressureDiagnosing headache type comments:Paroxysmal headache occurs in 51–80% of patientswith phaeochromocytoma. It is often severe, frontalor occipital and is generally described as either pulsatingor steady in quality. An important feature ofthe headache is its short duration: <15 minutes in50% and <1 hour in 70% of patients. Other featuresinclude apprehension and/or anxiety, often with asense of impending death, tremor, visual disturbances,abdominal or chest pain, nausea, vomitingand occasionally paraesthesia. The face can blanchor flush during the attack.The diagnosis is established by the demonstrationof increased excretion of catecholamines or catecholaminemetabolites, and can usually be securedby analysis of a single 24-hour urine sample collectedwhen the patient is hypertensive orsymptomatic.When hypertensive encephalopathy is present,headache is coded as 10.3.3 Headache attributed tohypertensive encephalopathy. When the diagnosis ofphaeochromocytoma has not yet been made, andhypertensive encephalopathy is not present, patientsmay meet the diagnostic criteria for 10.3.2 Headacheattributed to hypertensive crisis without hypertensiveencephalopathy.10.3.2 Headache attributed to hypertensive crisiswithout hypertensive encephalopathyDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:Diagnosing headache type (IHS 2003)110 ICHD-II1. bilateral2. pulsating quality3. precipitated by physical activityB. Hypertensive crisis defined as a paroxysmal risein systolic (to >160mmHg) and/or diastolic (to>120mmHg) blood pressure but no clinical featuresof hypertensive encephalopathyC. Headache develops during hypertensive crisisD. Headache resolves within 1 hour after normalisationof blood pressureE. Appropriate investigations have ruled out vasopressortoxins or medications as causative factorsDiagnosing headache type comment:Paroxysmal hypertension may occur in associationwith failure of baroreceptor reflexes (after carotidendarterectomy or subsequent to irradiation of theneck) or in patients with enterochromaffin celltumours.10.3.3 Headache attributed to hypertensiveencephalopathyDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. diffuse pain2. pulsating quality3. aggravated by physical activityB. Persistent blood pressure elevation to >160/100mmHg with at least two of the following:1. confusion2. reduced level of consciousness3. visual disturbances (other than those of typicalmigraine aura) including blindness4. seizuresC. Headache develops in close temporal relation toblood pressure elevationD. Headache resolves within 3 months after effectivetreatment and control of hypertensionE. Other causes of the neurological symptoms havebeen excludedDiagnosing headache type comments:Hypertensive encephalopathy is thought to occurwhen compensatory cerebrovascular vasoconstrictioncan no longer prevent cerebral hyperperfusionas blood pressure rises. As normal cerebral autoregulationof blood flow is overwhelmed, endothelialpermeability increases and cerebral oedema occurs.On MRI, this is often most prominent in the parietooccipitalwhite matter.Although hypertensive encephalopathy inpatients with chronic arterial hypertension is usuallyaccompanied by a diastolic blood pressure of >120mmHg, and by grade 3 or 4 hypertensive retinopathy(Keith-Wagner classification), previously normotensiveindividuals may develop signs ofencephalopathy with blood pressures as low as160/100 mmHg. Hypertensive retinopathy may notbe present at the time of clinical presentation.Any cause of hypertension, including phaeochromocytomaand ingestion of vasopressor toxins, canlead to hypertensive encephalopathy.10.3.4 Headache attributed to pre-eclampsiaDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. pulsating quality3. aggravated by physical activityB. Pregnancy or puerperium (up to 7 days postpartum),and pre-eclampsia defined by both ofthe following:1. hypertension (>140/90mmHg) documentedon two blood pressure readings at least 4hours apart2. urinary protein excretion >0.3 g per 24 hoursC. Headache develops during periods of high bloodpressureD. Headache resolves within 7 days after effectivetreatment of hypertensionE. Appropriate investigations have ruled out vasopressortoxins, medications or phaeochromocytomaas causative factorsDiagnosing headache type comment:A placenta appears essential for the developmentof pre-eclampsia. Pre-eclampsia is a multi-systemdisorder with various forms. In addition to hypertensionand proteinuria, tissue oedema, thrombocytopeniaand abnormalities in liver function canoccur. Pre-eclampsia appears to involve a strongmaternal inflammatory response, with broadimmunological systemic activity.10.3.5 Headache attributed to eclampsiaDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. pulsating quality3. aggravated by physical activityB. Pregnancy or puerperium (up to 4 weeks postpartum),and eclampsia defined by all of thefollowing:Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 1111. hypertension (>140/90mmHg) documentedon two blood pressure readings at least 4hours apart2. urinary protein excretion >0.3 g per 24 hours3. a seizure has occurredC. Headache develops during periods of high bloodpressureD. Headache resolves within 7 days after effectivetreatment of hypertensionE. Appropriate investigations have ruled out vasopressortoxins, medications or phaeochromocytomaas causative factorsF. Stroke has been excludedDiagnosing headache type comment:Case reports indicate that eclampsia can occur in thepuerperium as well as during pregnancy.10.3.6 Headache attributed to acute pressorresponse to an exogenous agentCoded elsewhere:8.1.6 Cocaine-induced headache.Diagnosing headache type diagnostic criteria:A. Headache, no typical characteristics known, fulfillingcriteria C and DB. An appropriate agent or toxin has been administeredor ingested and an acute rise in blood pressurehas occurredC. Headache develops in close temporal relation tothe acute rise in blood pressureD. Headache resolves within 24 hours after normalisationof blood pressureE. No other mechanism for the headache isapparentDiagnosing headache type comments:Apart from cocaine, agents that can produce acuteelevations of blood pressure include sympathomimeticsand amphetamines, and monoamineoxidase inhibitors when interactions with tyraminecontainingfoods occur.There is insufficient evidence to set criteria forhow large an elevation in blood pressure is requiredto produce headache, and this may vary from personto person. Criterion D is arbitrary, but included toincrease the specificity of the diagnostic criteria.10.4 Headache attributed to hypothyroidismDiagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. bilateral2. non-pulsatile3. continuousB. Hypothyroidism is demonstrated by appropriateinvestigationsC. Headache develops within 2 months after othersymptoms of hypothyroidism become evidentD. Headache resolves within 2 months after effectivetreatment of hypothyroidismDiagnosing headache type comment:It has been estimated that approximately 30% ofpatients with hypothyroidism suffer from headache.Its mechanism is unclear. There is a female preponderanceand often a history of migraine in childhood.Headache attributed to hypothyroidism is notassociated with nausea or vomiting.10.5 Headache attributed to fastingCoded elsewhere:Hypoglycaemia-induced migraine is coded accordingto subtype under 1. Migraine, with hypoglycaemiaconsidered as a precipitating factor.Diagnosing headache type diagnostic criteria:A. Headache with at least one of the following characteristicsand fulfilling criteria C and D:1. frontal location2. diffuse pain3. non-pulsating quality4. mild or moderate intensityB. The patient has fasted for >16 hoursC. Headache develops during fastingD. Headache resolves within 72 hours after resumptionof food intakeDiagnosing headache type comments:Headache with fasting is significantly more commonin individuals with a prior history of headache. Inthose individuals with a prior history of migraine,the headache may resemble 1.1 Migraine withoutaura.The likelihood of headache developing as a resultof a fast increases with the duration of the fast.The headache associated with fasting does notappear to be related to duration of sleep, to caffeinewithdrawal or to hypoglycaemia. Althoughheadache may occur under conditions of hypoglycaemia-induced brain dysfunction, there is no conclusiveevidence to support a causal association.Fasting headache can occur in the absence of hypoglycaemia,insulin-induced hypoglycaemia doesnot precipitate headache in migraine sufferers, andDiagnosing headache type (IHS 2003)112 ICHD-IIheadache is not a complaint of patients presenting tothe emergency department with symptomatic hypoglycaemia.Well-controlled studies are needed todemonstrate a causal relationship, if one exists.10.6 Cardiac cephalalgiaDiagnosing headache type diagnostic criteria:A. Headache, which may be severe, aggravated byexertion and accompanied by nausea and fulfillingcriteria C and DB. Acute myocardial ischaemia has occurredC. Headache develops concomitantly with acutemyocardial ischaemiaD. Headache resolves and does not recur after effectivemedical therapy for myocardial ischaemia orcoronary revascularisationDiagnosing headache type comment:Diagnosis must include careful documentation ofheadache and simultaneous cardiac ischaemiaduring treadmill or nuclear cardiac stress testing.Failure to recognise and correctly diagnose 10.6Cardiac cephalalgia can have grave consequences.Therefore, distinguishing this disorder from 1.1Migraine without aura is of crucial importance,particularly since vasoconstrictor medications (eg,triptans, ergots) are indicated in the treatment ofmigraine but contraindicated in patients withischaemic heart disease. Both disorders can producesevere head pain accompanied by nausea and bothdisorders can be triggered by exertion. Migraine-likeheadache may be triggered by angina treatment suchas nitroglycerine.10.7 Headache attributed to other disorder ofhomoeostasisDiagnosing headache type diagnostic criteria:A. Headache fulfilling criteria C and DB. Evidence of a disorder of homoeostasis otherthan those described aboveC. Headache develops within 2 months after onsetof the disorder, and other evidence exists that thedisorder can cause headacheD. Headache resolves within 3 months after relieffrom the disorder of homoeostasisBibliography10.1.1 High-altitude headache; 10.1.3 Sleep apnoeaheadacheAldrich MS, Chauncey JB. Are morning headaches part ofobstructive sleep apnea syndrome? Arch Intern Med 1990;150:1265–7.Appenzeller O. Altitude Headache. Headache 1972; 12:126–9.Ginsberg MD. Carbon monoxide intoxication: clinical features,neuropathology and mechanisms of injury. J ToxicolClin Toxicol 1985; 23:281–8.Heckerling PS, Leikiin JB, Maturen A, Perkins JT. Predictorsof occult carbon monoxide poisoning in patients withheadache and dizziness. Ann Intern Med 1987; 107:174–6.Jozefowicz RF. Neurologic manifestations of pulmonarydisease. Neurologic Clinics 1989; 7:605–16.Lipton RB, Mazer C, Newman LC, Solomon S. Sumatriptanrelieves migraine-like headaches associated with carbonmonoxide exposure. Headache 1997; 37:392–5.Loh NK, Dinner DS, Foldvary DO, Skobieranda F, Yew WW.Do patients with obstructive sleep apnea wake up withheadaches? Arch Intern Med 1999; 159:1765–8.Poceta JS, Dalessio DJ. Identification and treatment of sleepapnea in patients with chronic headache. Headache 1995;35:586–9.Porcelli J, Gugelchuk G. A trek to the top: A review of acutemountain sickness. J Amer Osteopath Assoc 1995;95:718–20.Silber E, Sonnenberg P, Collier DJ, Pollard A, Murdoch DR,Goadsby PJ. Clinical features of headache at altitude: aprospective study. Neurology 2003; 60:1167–71.10.1.2 Diving headacheCheshire WP, Ott MC Jr. Headache in divers. Headache 2001;41:235–47.Edmonds RC, Greene ER, Schoene RB et al. Diving andsubaquative medicine. 3rd Ed. Oxford: Butterworth-Heinemann;1992: pp 404–6.Sliwka U, Kransney JA, Simon SG et al. Effects of sustainedlow-level elevations of carbon dioxide on cerebral bloodflow and autoregulation of the intracerebral arteries inhumans. Aviat Space Environ Med 1998; 69:299–306.10.2 Dialysis headacheAntoniazzi AL, Bigal ME, Bordini CA, Speciali JG. Headacheassociated with dialysis. The IHS criteria revisited. Cephalalgia2003; 23:146–9.Jameson MD, Wiegmann TB. Principles, uses, and complicationsof hemodialysis. Medical Clinics of North America1990; 74:945–60.10.3 Headache attributed to arterial hypertensionDodick DW. Recurrent short-lasting headache associated withparoxysmal hypertension: a clonidine-responsive syndrome.Cephalalgia 2000; 20:509–14.Gus M, Fuchs FD, Pimentel M, Rosa D, Melo AG, Moreira LB.Behavior of ambulatory blood pressure surroundingepisodes of headache in mildly hypertensive patients. ArchIntern Med 2001; 161:252–5.Kruszewski P, Bieniaszewski L, Neubauer J, Krupa-Wojciechowska B. Headache in patients with mild tomoderate hypertension is generally not associated withsimultaneous blood pressure elevation. J Hypertension2000; 18:437–44.Lance JW, Hinterberger H. Symptom of pheochromocytomawith particular reference to headache, correlated with catecholamineproduction. Arch Neurol 1976; 33:281–8.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 113Land SH, Donovan T. Pre-eclampsia and eclampsia headache:classification recommendation [letter]. Cephalalgia 1999;19:67–9.Loh KC, Shlossberg AH, Abbott EC, Salisbury SR, Tan MH.Phaeochromocytoma: a ten-year survey. Quart J Med 1997;90:51–60.Mannelli M, Ianni L, Cilotti A, Conti A. Pheochromocytomain Italy: Amulticentric retrospective study. Eur J Endocrinol1999; 141:619–624.Thomas JE, Rooke ED, Kvale WF. The neurologists experiencewith pheochromocytoma. JAMA 1966; 197:754–58.Vaughan CJ, Delanty N. Hypertensive emergencies. Lancet2000; 356:411–17.Walker JJ. Pre-eclampsia. Lancet 2000; 56:1260–65.Weiss NS. Relation of high blood pressure to headache, epistaxis,and selected other symptoms. The United StatesHealth Examination Survey of Adults. N Engl J Med. 1972;287:631–3.Zampaglione B, Pascale C, Marchisio M, Cavallo-Perin P.Hypertensive urgencies and emergencies. Prevalence andclinical presentation. Hypertension 1996; 27:144–7.10.4 Headache attributed to hypothyroidismAiraghi L, Catania A. Endocrine headache. In: Seminars inheadache management. Neuroendocrinological aspects ofheadache, vol 4, number 4. B.C.Decker Inc, 1999: pp 1–15.Amy JR. Tests of thyroid function in chronic headachepatients. Headache 1987; 27:351–3.Arafah BM, Prunty D, Ybarra J, Hlavin ML, Selman WR. Thedominant role of increased intrasellar pressure in thepathogenesis hypopituitarism, hyperprolactinemia, andheadache in patients with pituitary adenomas. J ClinEndocrinol Metab 2000; 85:1789–93.Fenichel NM. Chronic headache due to masked hypothyroidism.Ann Intern Med 1948; 29:456–60.Moreau T. Headache in hypothyroidism. Prevalence andoutcome under thyroid hormone therapy. Cephalalgia 1988;18:687–9.10.5 Headache attributed to fastingDalton K. Food intake prior to migraine attacks. Study of 2,313spontaneous attacks. Headache 1975; 15:188–93.Dexter JD, Roberts J, Byer JA. The five hour glucose tolerancetest and effect of low sucrose diet in migraine. Headache1978; 18:91–4.Malouf R, Brust JCM. Hypoglycemia: causes, neurologicalmanifestations, and outcome. Ann Neurol 1985; 17:421–30.Mosek AC, Korczyn AD. Yom Kippur Headache. Neurology1995;45:1953–5.Pearce J. Insulin induced hypoglycaemia in migraine. J NeurolNeurosurg Psychiat 1971; 34:154–6.Service FJ. Hypoglycemic disorders. In: Wyngaarden JB,Smith LH, Bennett JC, eds. Cecil’s textbook of medicine,18th ed. Philadelphia: WB Saunders, 1992:1310–7.10.6 Cardiac cephalalgiaBlacky RA, Rittlemeyer JT, Wallace MR. Headache angina. AmJ Cardiol 1987; 60:730.Bowen J, Oppenheimer G. Headache as a presentation ofangina: reproduction of symptoms during angioplasty.Headache 1993; 33:238–239.Fleetcroft R, Maddocks JL. Headache due to ischaemic heartdisease. J R Soc Med 1985; 78:676.Grace A, Horgan J, Breathnach K, Staunton H. Anginalheadache and its basis. Cephalalgia 1997; 17:195–6.Lefkowitz D, Biller J. Bregmatic headache as a manifestationof myocardial ischemia. Arch Neurol 1982; 39:130.Lipton RB, Lowenkopf T, Bajwa ZH, Leckie RS, Ribeiro S,Newman LC, Greenberg MA. Cardiac cephalgia: a treatableform of exertional headache. Neurology 1997; 49:813–6.Vernay D, Deffond D, Fraysse P, Dordain G. Walk headache:an unusual manifestation of ischemic heart disease.Headache 1989; 29:350–1.Diagnosing headache type (IHS 2003)114 ICHD-II11. Headache or facial pain attributed todisorder of cranium, neck, eyes, ears, nose,sinuses, teeth, mouth or other facial orcranial structures11.1 Headache attributed to disorder of cranialbone11.2 Headache attributed to disorder of neck11.2.1 Cervicogenic headache11.2.2 Headache attributed to retropharyngealtendonitis11.2.3 Headache attributed to craniocervicaldystonia11.3 Headache attributed to disorder of eyes11.3.1 Headache attributed to acute glaucoma11.3.2 Headache attributed to refractive errors11.3.3 Headache attributed to heterophoria orheterotropia (latent or manifest squint)11.3.4 Headache attributed to ocularinflammatory disorder11.4 Headache attributed to disorder of ears11.5 Headache attributed to rhinosinusitis11.6 Headache attributed to disorder of teeth, jawsor related structures11.7 Headache or facial pain attributed totemporomandibular joint (TMJ) disorder11.8 Headache attributed to other disorder ofcranium, neck, eyes, ears, nose, sinuses, teeth,mouth or other facial or cervical structuresCoded elsewhere:Headaches that are due to head or neck trauma areclassified under 5. Headache attributed to head and/orneck trauma. Neuralgiform headaches are classifiedunder 13. Cranial neuralgias and central causes of facialpain.General commentPrimary or secondary headache or both?When a new headache occurs for the first time inclose temporal relation to a craniocervical disorder,it is coded as a secondary headache attributed to thatdisorder. This is also true if the headache has thecharacteristics of migraine, tension-type headache orcluster headache. When a pre-existing primaryheadache is made worse in close temporal relationto a craniocervical disorder, there are two possibilities,and judgment is required. The patient caneither be given only the diagnosis of the pre-existingprimary headache or be given both this diagnosisand the diagnosis of headache attributed to the craniocervicaldisorder. Factors that support adding thelatter diagnosis are: a very close temporal relationto the craniocervical disorder, a marked worseningof the pre-existing headache, very good evidencethat the craniocervical disorder can aggravate theprimary headache and, finally, improvement orresolution of the headache after relief from the craniocervicaldisorder.Definite, probable or chronic?Adiagnosis of Headache or facial pain attributed to disorderof cranium, neck, eyes, ears, nose, sinuses, teeth,mouth or other facial or cranial structures usuallybecomes definite only when the headache resolvesor greatly improves after effective treatment or spontaneousremission of the craniocervical disorder. Ifthis disorder cannot be treated effectively or does notremit spontaneously, or when there has been insufficienttime for this to happen, a diagnosis ofHeadache probably attributed to the [specified] craniocervicaldisorder is usually applied.If the craniocervical disorder is effectively treatedor remits spontaneously but headache does notresolve or markedly improve after 3 months, the persistingheadache has other mechanisms. Nevertheless,A11.9 Chronic post-craniocervical disorder headacheis described in the appendix. Headaches meetingthese criteria exist but have been poorly studied andthe appendix entry is intended to stimulate furtherresearch into such headaches and their mechanisms.IntroductionDisorders of the cervical spine and of other structuresof the neck and head have not infrequentlybeen regarded as the commonest causes of headache,since many headaches originate from the cervical,nuchal or occipital regions or are localised there.Moreover, degenerative changes in the cervical spinecan be found in virtually all people over 40 years ofage. The localisation of pain and the X-ray detectionof degenerative changes have been plausible reasonsfor regarding the cervical spine as the most frequentcause of headaches. However, large-scale controlledstudies have shown that such changes are just aswidespread among individuals who do not sufferfrom headaches. Spondylosis or osteochondrosiscannot therefore be seen as the explanation ofheadaches. Asimilar situation applies to other widespreaddisorders: chronic sinusitis, temporomandibularjoint disorders and refractive errors ofthe eyes.Without specific criteria it would be possible forvirtually any type of headache to be classified asDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 115Headache or facial pain attributed to disorder of cranium,neck, eyes, ears, nose, sinuses, teeth, mouth or other facialor cranial structures, and this problem existed in thepast. It is not sufficient merely to list manifestationsof headaches in order to define them, since thesemanifestations are not unique. The purpose of thecriteria in this chapter is not to describe headachesin all their possible subforms, but rather to establishspecific causal relationships between headaches andfacial pain and the disorders of the cranium, neck,eyes, ears, nose, sinuses, teeth, mouth and otherfacial or cranial structures where these exist. For thisreason it has been necessary to identify strict specificoperational criteria for cervicogenic headache andother causes of headache described in this chapter. Itis not possible here to take account of diagnostic teststhat are unconfirmed or for which quality criteriahave not been investigated. Instead the aim of therevised criteria is to motivate as a future task thedevelopment of reliable and valid operational teststo establish specific causal relationships betweenheadaches and craniocervical disorders that are currentlyavailable only to a very limited extent.Headache disorders attributed to causes includedhere for the first time are 11.2.3 Headache attributed tocraniocervical dystonia and 11.3.4 Headache attributed toocular inflammatory disorders.11.1 Headache attributed to disorder of cranial boneDiagnosing headache type diagnostic criteria:A. Pain in one or more regions of the head or facefulfilling criteria C and DB. Clinical, laboratory and/or imaging evidence ofa lesion within the cranial bone known to be, orgenerally accepted as, a valid cause of headache1C. Pain develops in close temporal relation to and ismaximal over the bone lesionD. Pain resolves within 3 months after successfultreatment of the bone lesionNote:1. Most disorders of the skull (eg, congenital abnormalities,fractures, tumours, metastases) areusually not accompanied by headache. Exceptionsof importance are osteomyelitis, multiplemyeloma and Paget’s disease. Headache mayalso be caused by lesions of the mastoid, and bypetrositis.11.2 Headache attributed to disorder of neckDiagnosing headache type comment:Headache attributed to disorder of neck but not fulfillingthe criteria for any of 11.2.1 Cervicogenicheadache, 11.2.2 Headache attributed to retropharyngealtendonitis or 11.2.3 Headache attributed to craniocervicaldystonia is not sufficiently validated.11.2.1 Cervicogenic headacheDiagnosing headache type Diagnosing headache type previously used term:Cervical headacheCoded elsewhere:Headache causally associated with cervical myofascialtender spots is coded as 2.1.1 Infrequent episodictension-type headache associated with pericranial tenderness,2.2.1 Frequent episodic tension-type headacheassociated with pericranial tenderness or 2.3.1 Chronictension-type headache associated with pericranialtenderness.Diagnosing headache type diagnostic criteria:A. Pain, referred from a source in the neck and perceivedin one or more regions of the head and/orface, fulfilling criteria C and DB. Clinical, laboratory and/or imaging evidence ofa disorder or lesion within the cervical spine orsoft tissues of the neck known to be, or generallyaccepted as, a valid cause of headache1C. Evidence that the pain can be attributed to theneck disorder or lesion based on at least one ofthe following:1. demonstration of clinical signs that implicatea source of pain in the neck22. abolition of headache following diagnosticblockade of a cervical structure or its nervesupply using placebo- or other adequatecontrols3D. Pain resolves within 3 months after successfultreatment of the causative disorder or lesionNotes:1. Tumours, fractures, infections and rheumatoidarthritis of the upper cervical spine have not beenvalidated formally as causes of headache, but arenevertheless accepted as valid causes whendemonstrated to be so in individual cases. Cervicalspondylosis and osteochondritis are NOTaccepted as valid causes fulfilling criterion B.When myofascial tender spots are the cause, theheadache should be coded under 2. Tension-typeheadache.2. Clinical signs acceptable for criterion C1 musthave demonstrated reliability and validity. Thefuture task is the identification of such reliableand valid operational tests. Clinical features suchas neck pain, focal neck tenderness, history ofDiagnosing headache type (IHS 2003)116 ICHD-IIneck trauma, mechanical exacerbation of pain,unilaterality, coexisting shoulder pain, reducedrange of motion in the neck, nuchal onset, nausea,vomiting, photophobia etc are not unique to cervicogenicheadache. These may be features of cervicogenicheadache, but they do not define therelationship between the disorder and the sourceof the headache.3. Abolition of headache means complete relief ofheadache, indicated by a score of zero on a visualanalogue scale (VAS). Nevertheless, acceptable asfulfilling criterion C2 is ≥90% reduction in pain toa level of <5 on a 100-point VAS.11.2.2 Headache attributed to retropharyngealtendonitisDiagnosing headache type diagnostic criteria:A. Unilateral or bilateral non-pulsating pain in theback of the neck, radiating to the back of the heador to the whole head and fulfilling criteria C andD

B. Swollen prevertebral soft tissues, in adults measuring>7mm at the level between C1 and C4(special X-ray technique may be required)C. Pain is aggravated severely by bending the headbackwardsD. Pain is alleviated within 2 weeks of treatmentwith non-steroidal anti-inflammatory drugs intheir recommended dosesDiagnosing headache type comments:Body temperature and erythrocyte sedimentationrate (ESR) are usually elevated. Although retroflexionof the neck most consistently aggravates pain,this also usually happens with rotation and swallowing.The transverse processes of the upper threevertebrae are usually tender to palpation.In several cases amorphous calcific material hasbeen aspirated from the swollen prevertebral tissues.Thin calcification in prevertebral tissues is best seenon CT.Upper carotid dissection should be ruled out.11.2.3 Headache attributed to craniocervicaldystoniaDiagnosing headache type diagnostic criteria:A. Sensation of cramp, tension or pain in the neck,radiating to the back of the head or to the wholehead and fulfilling criteria C and DB. Abnormal movements or defective posture ofneck or head due to muscular hyperactivityC. Evidence that pain is attributed to muscularhyperactivity based on at least one of thefollowing:1. demonstration of clinical signs that implicatea source of pain in the hyperactive muscle (eg,pain is precipitated or exacerbated by musclecontraction, movements, sustained posture orexternal pressure)2. simultaneous onset of pain and muscularhyperactivityD. Pain resolves within 3 months after successfultreatment of the causative disorderDiagnosing headache type comment:Focal dystonias of the head and neck accompaniedby pain are pharyngeal dystonia, spasmodic torticollis,mandibular dystonia, lingual dystonia and acombination of the cranial and cervical dystonias(segmental craniocervical dystonia). Pain is causedby local contractions and secondary changes.11.3 Headache attributed to disorder of eyes11.3.1 Headache attributed to acute glaucomaDiagnosing headache type diagnostic criteria:A. Pain in the eye and behind or above it, fulfillingcriteria C and DB. Raised intraocular pressure, with at least one ofthe following:1. conjunctival injection2. clouding of cornea3. visual disturbancesC. Pain develops simultaneously with glaucomaD. Pain resolves within 72 hours of effective treatmentof glaucoma11.3.2 Headache attributed to refractive errorsDiagnosing headache type diagnostic criteria:A. Recurrent mild headache, frontal and in the eyesthemselves, fulfilling criteria C and DB. Uncorrected or miscorrected refractive error (eg,hyperopia, astigmatism, presbyopia, wearing ofincorrect glasses)C. Headache and eye pain first develop in closetemporal relation to the refractive error, areabsent on awakening and aggravated by prolongedvisual tasks at the distance or angle wherevision is impairedD. Headache and eye pain resolve within 7 days,and do not recur, after full correction of therefractive errorDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 11711.3.3 Headache attributed to heterophoria orheterotropia (latent or manifest squint)Diagnosing headache type diagnostic criteria:A. Recurrent non-pulsatile mild-to-moderate frontalheadache fulfilling criteria C and DB. Heterophoria or heterotropia has been demonstrated,with at least one of the following:1. intermittent blurred vision or diplopia2. difficulty in adjusting focus from near todistant objects or vice versaC. At least one of the following:1. headache develops or worsens during a visualtask, especially one that is tiring2. headache is relieved or improved on closingone eyeD. Headache resolves within 7 days, and does notrecur, after appropriate correction of vision11.3.4 Headache attributed to ocular inflammatorydisorderDiagnosing headache type diagnostic criteria:A. Pain in the eye and behind or around it, fulfillingcriteria C and DB. Ocular inflammation diagnosed by appropriateinvestigationsC. Headache develops during inflammationD. Headache resolves within 7 days after relief ofthe inflammatory disorderDiagnosing headache type comment:Ocular inflammation takes many forms, and maybe categorised variously by anatomical site (ie, iritis,cyclitis, choroiditis), by course (acute, subacute,chronic), by presumed cause (infectious agents thatare endogenous or exogenous, lens-related, traumatic),or by type of inflammation (granulomatous,non-granulomatous).11.4 Headache attributed to disorder of earsCoded elsewhere:Headache attributed to acoustic neuroma is codedas 7.4.2 Headache attributed directly to neoplasm.Headache attributed to a lesion, not of the ear, givingrise to referred otalgia is coded according to the siteand/or nature of the lesion.Diagnosing headache type diagnostic criteria:A. Headache accompanied by otalgia and fulfillingcriteria C and DB. Structural lesion of the ear diagnosed by appropriateinvestigationsC. Headache and otalgia develop in close temporalrelation to the structural lesionD. Headache and otalgia resolve simultaneouslywith remission or successful treatment of thestructural lesionDiagnosing headache type comments:There is no evidence that any pathology of the earcan cause headache without concomitant otalgia.Structural lesions of the pinna, external auditorycanal, tympanic membrane or middle ear may giverise to primary otalgia associated with headache.However, only about 50% of all cases of earacheare due to structural lesions of the external or middleear. Disorders outside this region may lead to referredotalgia as a result of radiation of pain into the earregion. Sensory fibres of the fifth, seventh, ninth andtenth cranial nerves project into the auricle, externalauditory canal, tympanic membrane and middle ear.For this reason referred pain from remote structurallesions in any of the anatomical regions to whichthese nerves project can be felt as referred otalgia.Since these are not disorders of the ear they arecoded elsewhere according to the site and/or natureof the lesion(s).11.5 Headache attributed to rhinosinusitisCoded elsewhere:‘Sinus headaches’Diagnosing headache type diagnostic criteria:A. Frontal headache accompanied by pain in one ormore regions of the face, ears or teeth and fulfillingcriteria C and DB. Clinical, nasal endoscopic, CT and/or MRIimaging and/or laboratory evidence of acute oracute-on-chronic rhinosinusitis1;2C. Headache and facial pain develop simultaneouslywith onset or acute exacerbation of rhinosinusitisD. Headache and/or facial pain resolve within 7days after remission or successful treatment ofacute or acute-on-chronic rhinosinusitisNotes:1. Clinical evidence may include purulence in thenasal cavity, nasal obstruction, hyposmia/anosmia and/or fever.2. Chronic sinusitis is not validated as a cause ofheadache or facial pain unless relapsing into anacute stage.Diagnosing headache type (IHS 2003)118 ICHD-IIDiagnosing headache type comments:Other conditions that are often considered to induceheadache are not sufficiently validated as causes ofheadache. These include deviation of nasal septum,hypertrophy of turbinates, atrophy of sinus membranesand mucosal contact. The last, however, isdefined in the appendix under A11.5.1 Mucosalcontact point headache.Migraine and tension-type headache are oftenconfused with 11.5 Headache attributed to rhinosinusitisbecause of similarity in location of the headache.Agroup of patients can be identified who have all ofthe features of 1.1 Migraine without aura and, additionally,concomitant clinical features such as facialpain, nasal congestion and headache triggered byweather changes. None of these patients have purulentnasal discharge or other features diagnostic ofacute rhinosinusitis. Therefore it is necessary to differentiate11.5 Headache attributed to rhinosinusitisfrom so-called ‘sinus headaches’, a commonly-madebut non-specific diagnosis. Most such cases fulfil thecriteria for 1.1 Migraine without aura, with headacheeither accompanied by prominent autonomic symptomsin the nose or triggered by nasal changes.11.6 Headache attributed to disorder of teeth, jaws orrelated structuresDiagnosing headache type diagnostic criteria:A. Headache accompanied by pain in the teethand/or jaw(s) and fulfilling criteria C and DB. Evidence of disorder of teeth, jaws or relatedstructuresC. Headache and pain in teeth and/or jaw(s)develop in close temporal relation to the disorderD. Headache and pain in teeth and/or jaw(s) resolvewithin 3 months after successful treatment of thedisorderDiagnosing headache type comment:Disorders of the teeth usually cause toothacheand/or facial pain, and those causing headache arerare. Pain from the teeth may be referred, however,and cause diffuse headache. The most commoncause of headache is periodontitis or pericoronitis asthe result of infection or traumatic irritation arounda partially-erupted lower wisdom tooth.11.7 Headache or facial pain attributed totemporomandibular joint (TMJ) disorderDiagnosing headache type diagnostic criteria:A. Recurrent pain in one or more regions of the headand/or face fulfilling criteria C and DB. X-ray, MRI and/or bone scintigraphy demonstrateTMJ disorderC. Evidence that pain can be attributed to the TMJdisorder, based on at least one of the following:1. pain is precipitated by jaw movements and/orchewing of hard or tough food2. reduced range of or irregular jaw opening3. noise from one or both TMJs during jaw movements4. tenderness of the joint capsule(s) of one orboth TMJsD. Headache resolves within 3 months, and doesnot recur, after successful treatment of the TMJdisorderDiagnosing headache type comment:Pain from the temporomandibular joint or relatedtissues is common. It is due to the so-called temporomandibularjoint disorders (eg, disk displacements,osteoarthritis, joint hypermobility) orrheumatoid arthritis, and may be associated withmyofascial pain and headache.11.8 Headache attributed to other disorder of cranium,neck, eyes, ears, nose, sinuses, teeth, mouth or otherfacial or cervical structuresDiagnosing headache type diagnostic criteria:A. Headache, with or without pain in one or moreregions of the face, fulfilling criteria C and DB. Evidence of disorder, other than those describedabove, of cranium, neck, eyes, ears, nose, sinuses,teeth, mouth or other facial or cervical structureC. Headache develops in close temporal relation to,or other evidence exists of a causal relationshipwith, the disorder of cranium, neck, eyes, ears,nose, sinuses, teeth, mouth or other facial or cervicalstructureD. Headache resolves within 3 months after successfultreatment of the disorderBibliography11.1 Headache attributed to disorder of cranialboneBhatoe HS, Deshpande GU. Primary cranial Ewing’s sarcoma.Br J Neurosurg 1998; 12:165–9.Hayashi T, Kuroshima Y, Yoshida K, Kawase T, Ikeda E, MukaiM. Primary osteosarcoma of the sphenoid bone with extensiveperiosteal extension – case report. Neurol Med Chir(Tokyo) 2000; 40:419–22.Scherer A, Engelbrecht V, Nawatny J, Messing-Junger M,Reifenberger G, Modder U. MRI of the cerebellopontineangle in patients with cleidocranial dysostosis. RofoFortschr Geb Rontgenstr Neuen Bildgeb Verfahr 2001;173:315–8.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 11911.2 Headache attributed to disorder of neckAntonaci F, Fredriksen TA, Sjaastad O. Cervicogenicheadache: clinical presentation, diagnostic criteria, and differentialdiagnosis. Curr Pain Headache Rep 2001; 5:387–92.Antonaci F, Ghirmai S, Bono G, Sandrini G, Nappi G. Cervicogenicheadache: evaluation of the original diagnosticcriteria. Cephalalgia 2001; 21:573–83.Bogduk N, Corrigan B, Kelly P, Schneider G, Farr R. CervicalHeadache. Med J Aust 1985; 143:202–207.Bogduk N. Cervicogenic headache: anatomic basis and pathophysiologicmechanisms. Curr Pain Headache Rep 2001;5:382–6.Bogduk N. Headache and the neck. In: Goadsby PJ, SilbersteinSD (eds): Headache. Boston, Butterworth-Heinemann, 1997,pp. 369–381.Fredriksen TA, Sjaastad O. Cervicogenic headache: currentconcepts of pathogenesis related to anatomical structure.Clin Exp Rheumatol 2000;18(2 Suppl 19):S16–8.Göbel H, Edmeads JG. Disorders of the skull and cervicalspine. In: Olesen J, Tfelt-Hansen P, Welch KMA. TheHeadaches. 2nd edition. Lippincott Williams & Wilkins,Philadelphia; 2000: pp. 891–898.Göbel H. Die Kopfschmerzen. Ursachen, Mechanismen, Diagnostikund Therapie in der Praxis. Springer Verlag, Berlin,Heidelberg, New York, London, Paris, Tokyo, Hong Kong,Barcelona, Budapest; 1997: pp. 1–901.Lance JW, Anthony M. Neck-tongue syndrome on suddenturning of the head. J Neurol Neurosurg Psychiat 1980;43:97–101.Leone M, D’Amico D, Grazzi L, Attanasia A, Bussone G.Cervicogenic headache: a critical review of the current diagnosticcriteria. Pain 1998; 78:1–5.Leone M, D’Amico D, Moschiano F, Farinotti M, Fillipini G,Bussone G. Possible identification of cervicogenic headacheamong patients with migraine: an analysis of 374headaches. Headache 1995, 35:461–4.Lord S, Barnsley L, Wallis B, Bogduk N. Third occipitalheadache: a prevalence study. J Neurol Neurosurg Psychiat1994, 57:1187–90.Lord SM, Bogduk N. The cervical synovial joints as sources ofpost-traumatic headache. J Musculoskel Pain 1996, 4:81–94.Poughias L, Kruszewski P, Inan L. Cervicogenic headache: aclinical review with special emphasis on therapy. FunctNeurol 1997; 12:305–17.Sjaastad O, Fredriksen TA, Stolt-Nielsen A, Salvesen R, JansenJ, Pareja JA, Sjaastad O, Fredriksen TA. Cervicogenicheadache: the importance of sticking to the criteria. FunctNeurol 2002; 17:35–6.11.2.2 Retropharyngeal tendonitisEastwood JD, Hudgins PA, Malone D. Retropharyngeal effusionin acute calcific prevertebral tendonitis: diagnosis withCT and MR imaging. Am J Neuroradiol 1998; 19:1789–92.Ekbom K, Torhall J, Annell K, Traff J. Magnetic resonanceimaging in retropharyngeal tendonitis. Cephalalgia 1994;14:266–9.Pearce JM. Longus cervicis colli ‘myositis’ (syn: retropharyngealtendonitis). J Neurol Neurosurg Psychiat 1996;61:324–9.Sarkozi J, Fam AG. Acute calcific retropharyngeal tendonitis:an unusual cause of neck pain. Arthritis Rheum 1984;27:708–10.11.2.3 Craniocervical dystoniaCsala B, Deuschl G. Craniocervical dystonia. Pragmaticgeneral concept or nosologic entity? Nervenarzt 1994;65:75–94.Friedman J, Standaert DG. Dystonia and its disorders. NeurolClin 2001; 19:681–705.Göbel H, Deuschl G. Dauerkontraktionen kranialer oderzervikaler Muskeln. Münchener Medizinische Wochenschrift1997; 139:456–8.Göbel H, Heinze A, Heinze-Kuhn K, Austermann K. Botulinumtoxin A in the treatment of headache syndromes andpericranial pain syndromes. Pain 2001; 91:195–9.Markham CH. The dystonias. Curr Opin Neurol Neurosurg1992; 5:301–7.11.3 Headache attributed to disorder of eyesDaroff RB. Ocular causes of headache. Headache 1998;38:661–7.Daum KM, Good G, Tijerina L. Symptoms in video displayterminal operators and the presence of small refractiveerrors. J Am Optom Assoc 1988; 59:691–7.Gerling J, Janknecht P, Kommerell G. Orbital pain in opticneuritis and anterior ischemic optic neuropathy. Neuro-Ophthalmology 1998; 19:93–99.Göbel H, Martin TJ. Ocular disorders. In: Olesen J, Tfelt-Hansen P, Welch KMA. The Headaches. 2nd edition. LippincottWilliams & Wilkins, Philadelphia; 2000: pp. 899–904.Gordon GE, Chronicle EP, Rolan P. Why do we still not knowwhether refractive error causes headaches? Towards aframework for evidence based practice. OphthalmicPhysiol Opt 2001; 21:45–50.Lewis J, Fourman S. Subacute angle-closure glaucoma as acause of headache in the presence of a white eye. Headache1998; 38:684–6.McCluskey PJ, Lightman S, Watson PG et al. Posterior scleritis.Clinical features, systemic associations, and outcome ina large series of patients. Ophthalmology 1999; 106:2380–6.11.4 Headache attributed to disorder of ears; 11.5Headache attributed to rhinosinusitisAbu-Bakra M, Jones NS. Prevalence of nasal mucosal contactpoints in patients with facial pain compared with patientswithout facial pain. J Laryngol Otol 2001; 115:629–32.Blumenthal HJ. Headache and sinus disease. Headache 2001;41:883–8.Boes CJ, Swanson JW, Dodick DW. Chronic paroxysmal hemicraniapresenting as otalgia with a sensation of externalacoustic meatus obstruction: two cases and a pathophysiologichypothesis. Headache 1998; 38:787–91.Close LG, Aviv J. Headaches and disease of the nose andparanasal sinuses. Semin Neurol 1997; 17:351–4.De Vuyst D, De Schepper AM, Parizel PM. Chronic cocaineabuse. JBR-BTR 2001; 84:60.Göbel H, Baloh RW. Disorders of ear, nose, and sinus. In:Olesen J, Tfelt-Hansen P, Welch KMA. The Headaches. 2ndedition. Lippincott Williams & Wilkins, Philadelphia; 2000:pp. 905–12.Diagnosing headache type (IHS 2003)120 ICHD-IIKenny TJ, Duncavage J, Bracikowski J, Yildirim A, Murray JJ,Tanner SB. Prospective analysis of sinus symptoms and correlationwith paranasal computed tomography scan. OtolaryngolHead Neck Surg 2001; 125:40–3.Lam DK, Lawrence HP, Tenenbaum HC. Aural symptoms intemporomandibular disorder patients attending a craniofacialpain unit. J Orofac Pain 2001; 15:146–57.Lanza DC, Kennedy DW. Adult rhinosinusitis defined. Reportof the Rhinosinusitis Task Force Committee of the AmericanAcademy of Otolaryngology Head and Neck Surgery.Otolaryngol Head Neck Surg 1997; 117:S1–S7.Levine HL. Patients with headache and visual disturbance: adifferentiation between migraine and sinus headache. ArchOtolaryngol Head Neck Surg 2000: 126:234–35.Murphy E, Merrill RL. Non-odontogenic toothache. J Ir DentAssoc 2001; 47(2):46–58.Pinto A, De Rossi SS, McQuone S, Sollecito TP. Nasal mucosalheadache presenting as orofacial pain: a review of the literatureand a case report. Oral Surg Oral Med Oral PatholOral Radiol Endod 2001; 92:180–3.Sandstrom M, Wilen J, Oftedal G, Hansson Mild K. Mobilephone use and subjective symptoms. Comparison of symptomsexperienced by users of analogue and digital mobilephones. Occup Med (Lond) 2001; 51:25–35.Seiden AM, Martin VT. Headache and the frontal sinus. OtolaryngolClin North Am 2001; 34:227–41.Sydbom A, Blomberg A, Parnia S, Stenfors N, Sandstrom T,Dahlen SE. Health effects of diesel exhaust emissions. EurRespir J 2001; 17:733–46.Tosun F, Gerek M, Ozkaptan Y. Nasal surgery for contact pointheadaches. Headache 2000; 40:237–40.West B, Jones NS. Endoscopy-negative, computed tomography-negative facial pain in a nasal clinic. Laryngoscope2001; 111(4 Pt 1):581–6.11.6 Headache attributed to disorder of teeth, jawsor related structuresAllen DT, Voytovich MC, Allen JC. Painful chewing andblindness: signs and symptoms of temporal arteritis. J AmDent Assoc. 2000; 131:1738–41.Ciancaglini R, Radaelli G. The relationship between headacheand symptoms of temporomandibular disorder in thegeneral population. J Dent 2001; 29:93–8.Egermark I, Carlsson GE, Magnusson T. A 20-year longitudinalstudy of subjective symptoms of temporomandibulardisorders from childhood to adulthood. Acta OdontolScand 2001; 59:40–8.Epstein JB, Caldwell J, Black G. The utility of panoramicimaging of the temporomandibular joint in patients withtemporomandibular disorders. Oral Surg Oral Med OralPathol Oral Radiol Endod 2001; 92:236–9.Henrikson T, Ekberg EC, Nilner M. Symptoms and signs oftemporomandibular disorders in girls with normal occlusionand Class II malocclusion. Acta Odontol Scand 1997;55:229–35.Ivanhoe CB, Lai JM, Francisco GE. Bruxism after brain injury:successful treatment with botulinum toxin-A. Arch PhysMed Rehabil 1997; 78:1272–3.Kirveskari P. Prediction of demand for treatment of temporomandibulardisorders. J Oral Rehabil 2001; 28:572–5.Magnusson T, Egermark I, Carlsson GE. A longitudinal epidemiologicstudy of signs and symptoms of temporomandibulardisorders from 15 to 35 years of age. J OrofacPain 2000; 14:310–9.Marcusson A, List T, Paulin G, Dworkin S. Temporomandibulardisorders in adults with repaired cleft lip andpalate: a comparison with controls. EOS 2001; 23:193–204.Sonnesen L, Bakke M, Solow B. Malocclusion traits and symptomsand signs of temporomandibular disorders in childrenwith severe malocclusion. Eur J Orthod 1998; 20:543–59.11.7 Temporomandibular joint disorderList T, Wahlund K, Larsson B. Psychosocial functioning anddental factors in adolescents with temporomandibular disorders:a case-control study. J Orofac Pain 2001; 15:218–27.Ciancaglini R, Radaelli G. The relationship between headacheand symptoms of temporomandibular disorder in thegeneral population. J Dent 2001; 29:93–8.Molina OF, dos Santos Junior J, Nelson SJ, Nowlin T. Profileof TMD and Bruxer compared to TMD and nonbruxerpatients regarding chief complaint, previous consultations,modes of therapy, and chronicity. Cranio 2000; 18:205–19.Ogus H. Degenerative disease of the temporomandibular jointand pain-dysfunction syndrome. J Roy Soc Med 1978;71:748–54.Jacome D. Primary yawning headache. Cephalalgia 2001;21:697–9.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 12112. Headache attributed to psychiatricdisorder12.1 Headache attributed to somatisation disorder12.2 Headache attributed to psychotic disorderCoded elsewhere:Headache attributed to substance-dependence,abuse or withdrawal, headache attributed to acuteintoxication and headache attributed to medicationoveruse are all coded under 8. Headache attributed toa substance or its withdrawal.General commentPrimary or secondary headache or both?When a new headache occurs for the first time in closetemporal relation to a psychiatric disorder, it is codedas a secondary headache attributed to that disorder.This is also true if the headache has the characteristicsof migraine, tension-type headache or clusterheadache. When a pre-existing primary headache ismade worse in close temporal relation to a psychiatricdisorder, there are two possibilities, and judgmentis required. The patient can either be given only thediagnosis of the pre-existing primary headache orbe given both this diagnosis and the diagnosis ofheadache attributed to the psychiatric disorder.Factors that support adding the latter diagnosis are:a very close temporal relation to the psychiatricdisorder, a marked worsening of the pre-existingheadache, very good evidence that the psychiatricdisorder can aggravate the primary headache and,finally, improvement or resolution of the headacheafter relief from the psychiatric disorder.Definite, probable or chronic?Adiagnosis of Headache attributed to psychiatric disorderusually becomes definite only when theheadache resolves or greatly improves after effectivetreatment or spontaneous remission of the psychiatricdisorder. If this disorder cannot be treated effectivelyor does not remit spontaneously, or whenthere has been insufficient time for this to happen, adiagnosis of Headache probably attributed to psychiatricdisorder is usually applied.Chronic headache attributed to and persistingafter resolution of a psychiatric disorder has not yetbeen described.IntroductionOverall, there is very limited evidence supportingpsychiatric causes of headache. Thus, the only diagnosticcategories included in this classification arethose rare cases in which a headache occurs in thecontext of a psychiatric condition that is known tobe symptomatically manifested by headache (eg, apatient who reports a headache associated with thedelusion that a metal plate has been surreptitiouslyinserted into his or her head, or headache that isa manifestation of somatisation disorder). The vastmajority of headaches that occur in association withpsychiatric disorders are not causally related to thembut instead represent comorbidity (perhaps reflectinga common biological substrate). Headache hasbeen reported to be comorbid with a number of psychiatricdisorders, including major depressive disorder,dysthymic disorder, panic disorder, generalisedanxiety disorder, somatoform disorders and adjustmentdisorders. In such cases, both a primaryheadache diagnosis and the comorbid psychiatricdiagnosis should be made.However, clinical experience suggests that, insome cases, headache occurring exclusively duringsome common psychiatric disorders such as majordepressive disorder, panic disorder, generalisedanxiety disorder and undifferentiated somatoformdisorder may best be considered as attributed tothese disorders. To encourage further research intothis area, criteria for headaches attributed to thesepsychiatric disorders have been included in theappendix.A headache diagnosis should heighten the clinician’sindex of suspicion for major depressive disorder,panic disorder and generalised anxiety disorder,and vice-versa. Furthermore, evidence suggests thatthe presence of a comorbid psychiatric disordertends to worsen the course of migraine and/ortension-type headache by increasing the frequencyand severity of headache and making it less responsiveto treatment. Thus, identification and treatmentof any comorbid psychiatric condition is importantfor the proper management of the headache. In childrenand adolescents, primary headache disorders(migraine, episodic tension-type and especiallychronic tension-type headache) are often comorbidwith psychiatric disorder. Sleep disorder, separationanxietydisorder, school phobia, adjustment disorderand other disorders usually first diagnosed ininfancy, childhood or adolescence (particularlyattention-deficit/hyperactivity disorder [ADHD],conduct disorder, learning disorder, enuresis, encopresis,tic) should be carefully looked for and treatedif found, considering their negative burden in disabilityand prognosis of paediatric headache.To ascertain whether a headache should be attributedto a psychiatric disorder, it is clearly importantDiagnosing headache type (IHS 2003)122 ICHD-IIfirst to determine whether or not there is a psychiatricdisorder present with the headache. Optimally,this entails conducting a psychiatric evaluationfor the presence of a psychiatric disorder. At aminimum, however, it is important to inquire aboutcommonly co-morbid psychiatric symptoms such asgeneralised anxiety, panic attacks and depression.12.1 Headache attributed to somatisation disorderDiagnosing headache type diagnostic criteria:A. Headache, no typical characteristics known, fulfillingcriterion CB. Presence of somatisation disorder fulfilling DSMIVcriteria:1. history of many physical complaints beginningbefore age 30 that occur over a period ofseveral years and result in treatment beingsought and/or in significant impairment insocial, occupational or other important areasof functioning2. at least four pain symptoms, two non-paingastrointestinal symptoms, one sexual orreproductive symptom and one pseudoneurologicalsymptom3. after appropriate investigation, each of thesesymptoms cannot be fully explained by aknown general medical condition or the directeffects of a substance or medication; or, if thereis a related medical condition, the complaintsor impairment are in excess of what would beexpected from the history, examination or laboratoryfindingsC. Headache is not attributed to another causeDiagnosing headache type comments:Somatisation disorder, as defined in DSM-IV, is apolysymptomatic disorder characterised by multiplerecurring pains and gastrointestinal, sexual andpseudoneurological symptoms occurring for aperiod of years with onset before age 30. Thesesymptoms are, by definition, considered to besomatoform: that is, they are complaints of physicalsymptoms suggestive of, but not fully explained by,a medical condition or the direct effect(s) of a substance.In the United States, it is found predominantlyin women, in whom the lifetime risk isestimated to be 2.0%, with a female/male ratio ofapproximately 10 : 1. This ratio is not as large in someother cultures (eg, in Greeks and Puerto Ricans).It should be noted that the symptom requirementlaid out in DSM-IV is quite extensive: a minimum ofeight somatoform symptoms must have occurredover the patient’s lifetime, each one severe enoughto result in the seeking of medical help or the takingof a medication (prescribed or over-the-counter), orto affect the person’s functioning (eg, causing misseddays at work). DSM-IV has set such a high thresholdin order to reduce false positives, most particularlythe possibility that the ‘unexplained’ symptomsare in fact part of a complex and as yet undiagnosedmedical condition with variable symptom presentationsuch as multiple sclerosis or systemic lupus erythematosus.Somatoform disorders with fewer thaneight symptoms are diagnosed in DSM-IV as Undifferentiatedsomatoform disorder. Because of the difficultyand uncertainty associated with this diagnosis,A12.6 Headache attributed to undifferentiated somatoformdisorder is included only in the appendix.To ascertain whether headache is part of the presentationof somatisation disorder, it is important toask whether the patient has a history of multiplesomatic complaints, since at any one time the patientmay be focused on one particular complaint. Considerthe following case scenario (from Yutzy, 2003):A 35-year-old woman presented with a complaintof extreme headaches, ‘like a knife being stuckthrough the back of my head into my eye,’ as wellas other headaches virtually every day. Aftermedical and neurological examinations failed tosuggest any specific aetiology for either headache,it was important to take a careful history of pastsymptoms. In this case, the woman also reporteda history of other pains, including abdominal painassociated at times with nausea and vomiting,periods of constipation followed by diarrhoeawhich had resulted in investigation for gallbladderand peptic ulcer disease with no significantfindings, and pain ‘in all of my joints’ but particularlyin her knees and her back that she said hadbeen diagnosed as degenerative arthritis at age 27years yet no deformities had developed since. Shehad had menstrual problems since menarche, withpain that put her to bed and excessive flow with‘big blue clots’, which had resolved only afterhysterectomy two years earlier at age 33 years.The mother of four, she reported a long history ofsexual problems including pain with intercourse.She had been told that she had a ‘tipped uterus’.Throughout her life, she was seldom orgasmic andhad not enjoyed sex ‘for years’. She reportedepisodes of blurred vision with ‘spots’ in front ofher eyes, which caused her to stop work, and otherepisodes when she just could not hear anything,‘like someone put their hands over my ears.’ Shealso reported periods of uncontrollable shakingand a feeling that she was losing control of herDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 123body, for which she had been investigated forseizures. She reported that, at times, she hadfeared having some serious medical disease but‘with all the work-ups I have had, I am sure theywould have found something by now.’As was evident after a complete medical history,the headaches were part of a much more involvedsyndrome. This woman had had multiple physicalcomplaints with onset before age 30 that had no adequatemedical explanation, were severe enough tocause her to seek medical attention and affecteda variety of organ systems meeting the DSM-IV criteriafor Somatization disorder (ie, at least four painsymptoms [headaches, abdominal pain, back painand knee pain], at least two non-pain gastrointestinalsymptoms [nausea, vomiting, diarrhoea andconstipation], at least one sexual or reproductivesymptom [pain on intercourse, excessive menstrualflow, loss of sexual enjoyment] and at least onepseudoneurological symptom [muffled hearing,uncontrollable shaking, blurred vision, spots invisual field]). Thus, her headaches would be correctlydiagnosed as 12.1 Headache attributed to somatisationdisorder.12.2 Headache attributed to psychotic disorderDiagnosing headache type previously used terms:Delusional headacheDiagnosing headache type diagnostic criteria:A. Headache, no typical characteristics known, fulfillingcriteria C–EB. Delusional belief about the presence and/or aetiologyof headache1 occurring in the context ofdelusional disorder, schizophrenia, major depressiveepisode with psychotic features, manicepisode with psychotic features or other psychoticdisorder fulfilling DSM-IV criteriaC. Headache occurs only when delusionalD. Headache resolves when delusions remitE. Headache is not attributed to another causeNote:1. For example, a patient’s false conviction that heor she has a brain tumour or intracranial massgiving rise to headache, which would fulfil DSMIVcriteria for Delusional disorder, somatic type.Diagnosing headache type comments:Delusions, as defined in DSM-IV, are false fixedbeliefs based on incorrect inference about reality thatare firmly held despite obvious proof to the contrary.Delusions, like any firmly-held belief, can be aboutvirtually anything. In 12.2 Headache attributed to psychoticdisorder, the delusion specifically involves thepresence of headache. In some instances, the delusionmay involve a false belief that a serious medicalcondition (eg, brain tumour) is present and is thecause of the headache, despite repeated and appropriateauthoritative reassurance that no suchmedical condition is present. In other cases, thecontent of the delusion may be more bizarre: forexample, a delusion that a transmitter has been surgicallyimplanted into one’s head and that the transmitteris the cause of the headache.Delusional headache is apparently very rare andno empirical data are available about this condition.Bibliography and referenceCurioso EP, Young WB, Shecter AL, Kaiser R. Psychiatriccomorbidity predicts outcome in chronic daily headachepatients. Neurology 1999; 52 (Suppl 2): A471.Canestri P, Galli F, Guidetti V, Tomaciello A. Chronic DailyHeadache in children and adolescents: a two years followup.Cephalalgia 2001; 21:288.Guidetti V, Galli F, Fabrizi P, Napoli L, Giannantoni AS, BruniO, Trillo S. Headache and psychiatric comorbidity: clinicalaspects and outcome in an 8-year follow-up study. Cephalalgia1998; 18:455–62.Lake A. Behavioral and nonpharmacologic treatments ofheadache. Med Clin North Am 2001; 85:1055–75.Marazzitti D, Toni C, Pedri S, Bonucelli U et al. Prevalence ofheadache syndromes in panic disorder. Int Clin Psychopharmacol1999; 14:247–251.Mitsikostas DD, Thomas AM. Comorbidity of headache anddepressive disorders. Cephalalgia 1999; 19:211–217.Pakalnis A, Greenberg G, Drake ME, Paolich J. Pediatricmigraine prophylaxis with divalproex. J Child Neurol 2001;16:731–4.Radat F, Sakh D, Lutz G, el Amrani M, Ferreri M, Bousser MG.Psychiatric comorbidity is related to headache induced bychronic substance use in migraineurs. Headache 1999;39:477–80.Radat F, Psychopathology and headache. Rev Neurol 2000;156 (Suppl 4):4S62–7.Yutzy S. Somatoform disorders. In: Tasman A, Kay J, LiebermanJA. Psychiatry, 2nd ed. Chichester: John Wiley and Sons2003: pp. 1419–20.Diagnosing headache type (IHS 2003)