Diagnosing headache type (beta)
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Diagnostic criteria are based on IHS criteria 2003
Older IHS diagnostic criteria
In 1988 the International Headache Society published criteriafor diagnosing headache type[1]. The main types of primary headache and how the diagnostic criteria are applied are listed below. Migraine without aura (MO) diagnostic criteriaA. At least five headacheattacks lasting 4 - 72 hours (untreated or unsuccessfully treated),which has at least two of the four following characteristics: 1. Unilateral location 2. Pulsating quality 3. Moderate or severe intensity (inhibits or prohibits dailyactivities) 4. Aggravated by walking stairs or similar routine physicalactivity B. During headache at least oneof the two following symptoms occur: 1. Phonophobia and photophobia 2. Nausea and/or vomiting Migraine with aura (MA) diagnostic criteriaA. At least two attacksfulfilling with at least three of the following: 1. One or more fully reversible aura symptoms indicating focalcerebral cortical and/or brain stem functions 2. At least one aura symptom develops gradually over more thanfour minutes, or two or more symptoms occur in succession 3. No aura symptom lasts more than 60 minutes; if more thanone aura symptom is present, accepted duration is proportionallyincreased 4. Headache follows aura with free interval of at least 60minutes (it may also simultaneously begin with the aura B. At least one of thefollowing aura features establishes a diagnosis of migraine withtypical aura: 1. Homonymous visual disturbance 2. Unilateral paresthesias and/or numbness 3. Unilateral weakness 4. Aphasia or unclassifiable speech difficulty Cluster HeadacheA. At least five attacks ofsevere unilateral orbital, supraorbital and/or temporal pain lasting 15to 180 minutes untreated, with one or more of the following signsoccurring on the same side as the pain 1. Conjunctival injection 2. Lacrimation 3. Nasal congestion 4. Rhinorrhoea 5. Forehead and facial sweating 6. Miosis 7. Ptosis 8. Eyelid oedema B . Frequency of attacks fromone every other day to eight per day Tension-Type HeadacheA. Headache lasting from 30minutes to seven days B. At least two of thefollowing criteria: 1. Pressing/tightening (non-pulsatile) quality 2. Mild or moderate intensity (may inhibit, but does notprohibit activity) 3. Bilateral location 4. No aggravation by walking, stairs or similar routinephysical activity C . Both of the following: 1. No nausea or vomiting (anorexia may occur) 2. Photophobia and phonophobia are absent, or one but not bothare present
Cervicogenic HeadacheA. Pain localised to the neckand occipital region. May project to forehead, orbital region, temples,vertex or ears B. Pain is precipitated oraggravated by special neck movements or sustained postures C . At least one of thefollowing: 1. Resistance to or limitation of passive neck movements 2. Changes in neck muscle contour, texture, tone or responseto active and passive stretching and contraction 3. Abnormal tenderness of neck muscles D. Radiological examinationreveals at least one of the following 1. Movement abnormalities in flexion/extension 2. Abnormal posture 3. Fractures, congenital abnormalities, bone tumours,rheumatoid arthritis or other distinct pathology (not spondylosis orosteochondrosis) Reference for diagnosing headache type:1 Headache classification committee of the IHS. Classificationand diagnostic criteria for headache disorders, cranial neuralgias andfacial pain. Cephalalgia 1988 8: 1-96. ( diagnosing headache type)
IHS criteria archive data 2003Part oneThe primary headachesMigraineTension-type headacheCluster headache and other trigeminal autonomic cephalalgiasOther primary headaches24 ICHD-II1. Migraine1.1 Migraine without aura1.2 Migraine with aura1.2.1 Typical aura with migraine headache1.2.2 Typical aura with non-migraine headache1.2.3 Typical aura without headache1.2.4 Familial hemiplegic migraine (FHM)1.2.5 Sporadic hemiplegic migraine1.2.6 Basilar-type migraine1.3 Childhood periodic syndromes that arecommonly precursors of migraine1.3.1 Cyclical vomiting1.3.2 Abdominal migraine1.3.3 Benign paroxysmal vertigo of childhood1.4 Retinal migraine1.5 Complications of migraine1.5.1 Chronic migraine1.5.2 Status migrainosus1.5.3 Persistent aura without infarction1.5.4 Migrainous infarction1.5.5 Migraine-triggered seizure1.6 Probable migraine1.6.1 Probable migraine without aura1.6.2 Probable migraine with aura1.6.5 Probable chronic migraineCoded elsewhere:Migraine-like headache secondary to another disorder(symptomatic migraine) is coded according to thedisorder.General commentPrimary or secondary headache or both?When a headache with migraine characteristicsoccurs for the first time in close temporal relation toanother disorder that is a known cause of headache,it is coded according to the causative disorder as asecondary headache. When pre-existing migraine ismade worse in close temporal relation to anotherdisorder that is a known cause of headache, thereare two possibilities, and judgment is required. Thepatient can either be given only the migraine diagnosisor be given both the migraine diagnosis and asecondary headache diagnosis according to the otherdisorder. Factors that support adding the latterdiagnosis are: a very close temporal relation to thedisorder, a marked worsening of the migraine, verygood evidence that the disorder can cause or aggravatemigraine, and improvement or resolution ofmigraine after relief from the disorder.IntroductionMigraine is a common disabling primary headachedisorder. Epidemiological studies have documentedits high prevalence and high socio-economic andpersonal impacts. It is now ranked by the WorldHealth Organization as number 19 among all diseasesworld-wide causing disability.Migraine can be divided into two major sub-types.1.1 Migraine without aura is a clinical syndrome characterisedby headache with specific features andassociated symptoms. 1.2 Migraine with aura is primarilycharacterised by the focal neurological symptomsthat usually precede or sometimes accompanythe headache. Some patients also experience a premonitoryphase, occurring hours or days before theheadache, and a headache resolution phase. Premonitoryand resolution symptoms include hyperactivity,hypoactivity, depression, craving forparticular foods, repetitive yawning and other lesstypical symptoms reported by some patients.When a patient fulfils criteria for more than onesubtype of migraine, all subtypes should be diagnosedand coded. For example, a patient who hasfrequent attacks with aura but also some attackswithout aura should be coded as 1.2 Migraine withaura and 1.1 Migraine without aura.1.1 Migraine without auraDiagnosing headache type previously used terms:Common migraine, hemicrania simplexDiagnosing headache type description:Recurrent headache disorder manifesting in attackslasting 472 hours. Typical characteristics of theheadache are unilateral location, pulsating quality,moderate or severe intensity, aggravation by routinephysical activity and association with nauseaand/or photophobia and phonophobia.Diagnosing headache type diagnostic criteria:A. At least 5 attacks1 fulfilling criteria BDB. Headache attacks lasting 472 hours (untreatedor unsuccessfully treated)2;3;4C. Headache has at least two of the followingcharacteristics:1. unilateral location5;62. pulsating quality73. moderate or severe pain intensity4. aggravation by or causing avoidance ofroutine physical activity (eg, walking or climbingstairs)D. During headache at least one of the following:Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 251. nausea and/or vomiting2. photophobia and phonophobia8E. Not attributed to another disorder9Notes:1. Differentiating between 1.1 Migraine without auraand 2.1 Infrequent episodic tension-type headachemay be difficult. Therefore at least 5 attacks arerequired. Individuals who otherwise meet criteriafor 1.1 Migraine without aura but have had fewerthan 5 attacks should be coded 1.6.1 Probablemigraine without aura.2. When the patient falls asleep during migraine andwakes up without it, duration of the attack is reckoneduntil the time of awakening.3. In children, attacks may last 172 hours (althoughthe evidence for untreated durations of less than2 hours in children requires corroboration byprospective diary studies).4. When attacks occur on ≥15 days/month for >3months, code as 1.1 Migraine without aura and as1.5.1 Chronic migraine.5. Migraine headache is commonly bilateral inyoung children; an adult pattern of unilateralpain usually emerges in late adolescence or earlyadult life.6. Migraine headache is usually frontotemporal.Occipital headache in children, whether unilateralor bilateral, is rare and calls for diagnosticcaution; many cases are attributable to structurallesions.7. Pulsating means throbbing or varying with theheartbeat.8. In young children, photophobia and phonophobiamay be inferred from their behaviour.9. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type comments:1.1 Migraine without aura is the commonest subtypeof migraine. It has a higher average attack frequencyand is usually more disabling than 1.2 Migraine withaura.Migraine without aura often has a strict menstrualrelationship. In contrast to the first edition of TheInternational Classification of Headache Disorders, thisedition gives criteria for A1.1.1 Pure menstrualmigraine and A1.1.2 Menstrually-related migraine, butin the appendix because of uncertainty over whetherthey should be regarded as separate entities.Very frequent migraine attacks are now distinguishedas 1.5.1 Chronic migraine provided that thereis no medication overuse. Migraine without aura isthe disease most prone to accelerate with frequentuse of symptomatic medication, resulting in a newheadache which is coded as 8.2 Medication-overuseheadache.Regional cerebral blood flow shows no changessuggestive of cortical spreading depression duringattacks of migraine without aura although bloodflow changes in the brainstem may occur, as maycortical changes secondary to pain activation. Thiscontrasts with the pathognomonic spreading oligaemiaof migraine with aura. In all likelihoodspreading depression is therefore not involved inmigraine without aura. On the other hand the messengermolecules nitric oxide (NO) and calcitoningene-related peptide (CGRP) are clearly involved.While the disease was previously regarded as primarilyvascular, the importance of sensitisation ofperivascular nerve terminals, and the possibility thatattacks may originate in the central nervous system,have gained increasing attention over the lastdecades. At the same time the circuitry of migrainepain and several aspects of neurotransmission in thissystem have been recognised. A significant contributionhas been made by the advent of the triptans,5HT1B/D receptor agonists. These drugs have remarkableefficacy in acute attacks and, in view of theirhigh receptor-specificity, their mechanism of actionprovides new insight into migraine mechanisms. Itis now clear that migraine without aura is a neurobiologicaldisorder and clinical as well as basicneuroscience currently advances our knowledge ofmigraine mechanisms at an increasing speed.1.2 Migraine with auraDiagnosing headache type previously used terms:Classic or classical migraine, ophthalmic, hemiparaesthetic,hemiplegic or aphasic migraine,migraine accompagnιe, complicated migraineCoded elsewhere:13.17 Ophthalmoplegic migraine.Diagnosing headache type description:Recurrent disorder manifesting in attacks ofreversible focal neurological symptoms that usuallydevelop gradually over 520 minutes and last forless than 60 minutes. Headache with the featuresof migraine without aura usually follows the auraDiagnosing headache type (IHS 2003)26 ICHD-IIsymptoms. Less commonly, headache lacks migrainousfeatures or is completely absent.Diagnosing headache type diagnostic criteria:A. At least 2 attacks fulfilling criterion BB. Migraine aura fulfilling criteria B and C for oneof the subforms 1.2.11.2.6C. Not attributed to another disorder1Note:1. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type comments:The aura is the complex of neurological symptomsthat occurs just before or at the onset of migraineheadache. Most patients with migraine have exclusivelyattacks without aura. Many patients who havefrequent attacks with aura also have attacks withoutaura (code as 1.2 Migraine with aura and 1.1 Migrainewithout aura).Premonitory symptoms occur hours to a day ortwo before a migraine attack (with or without aura).They include various combinations of fatigue, difficultyin concentrating, neck stiffness, sensitivity tolight or sound, nausea, blurred vision, yawning andpallor. The terms prodrome and warning symptoms arebest avoided because they are often mistakenly usedto include aura.The majority of migraine auras are associated withheadache fulfilling criteria for 1.1 Migraine withoutaura. For this reason the entity 1.2.1 Typical aura withmigraine headache has been singled out below.Migraine aura is sometimes associated with aheadache that does not fulfil criteria for 1.1 Migrainewithout aura and, in other cases, migraine aura mayoccur without headache. These two subforms arealso now distinguished.Aura with similar features has also been describedin association with other well-defined headachetypes, including cluster headache; the relationshipsbetween aura and headache are not fullyunderstood.Before or simultaneously with the onset of aurasymptoms, regional cerebral blood flow is decreasedin cortex corresponding to the clinically affected areaand often including an even wider area. Blood flowreduction usually starts posteriorly and spreadsanteriorly and is usually above the ischaemic threshold.After one to several hours, gradual transitioninto hyperaemia occurs in the same region. Corticalspreading depression of Leγo has been implicated.Systematic studies have demonstrated that manypatients with visual auras occasionally have symptomsin the extremities. Conversely patients withsymptoms in the extremities virtually always alsosuffer visual aura symptoms. A distinction betweenmigraine with visual aura and hemiparaestheticmigraine is probably artificial and therefore is notrecognised in this classification. Patients with motorweakness are classified separately because of thedominantly inherited form, 1.2.4 Familial hemiplegicmigraine, and because of clinical differences. Thegenetic relationship between migraine with auraand familial hemiplegic migraine has not beenestablished.The previously-defined syndromes migraine withprolonged aura and migraine with acute-onset aura havebeen abandoned. The great majority of patients withsuch attacks have other attacks that fulfil criteria forone of the subforms of 1.2 Migraine with aura andshould be coded to that diagnosis. The rest shouldbe coded to 1.6.2 Probable migraine with aura, specifyingthe atypical feature (prolonged aura or acuteonsetaura) in parenthesis.1.2.1 Typical aura with migraine headacheDiagnosing headache type description:Typical aura consisting of visual and/or sensoryand/or speech symptoms. Gradual development,duration no longer than one hour, a mix of positiveand negative features and complete reversibilitycharacterise the aura which is associated with aheadache fulfilling criteria for 1.1 Migraine withoutaura.Diagnosing headache type diagnostic criteria:A. At least 2 attacks fulfilling criteria BDB. Aura consisting of at least one of the following,but no motor weakness:1. fully reversible visual symptoms includingpositive features (eg, flickering lights, spotsor lines) and/or negative features (ie, loss ofvision)2. fully reversible sensory symptoms includingpositive features (ie, pins and needles) and/ornegative features (ie, numbness)3. fully reversible dysphasic speech disturbanceC. At least two of the following:1. homonymous visual symptoms1 and/or unilateralsensory symptomsDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 272. at least one aura symptom develops graduallyover ≥5 minutes and/or different aura symptomsoccur in succession over ≥5 minutes3. each symptom lasts ≥5 and £60 minutesD. Headache fulfilling criteria BD for 1.1 Migrainewithout aura begins during the aura or followsaura within 60 minutesE. Not attributed to another disorder2Notes:1. Additional loss or blurring of central vision mayoccur.2. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type comments:This is the most common migraine syndrome associatedwith aura. The diagnosis is usually evidentafter a careful history alone though there are rare secondarymimics including carotid dissection, arteriovenousmalformation and seizure.Visual aura is the most common type of aura, oftenpresenting as a fortification spectrum, ie, a zigzagfigure near the point of fixation that may graduallyspread right or left and assume a laterally convexshape with an angulated scintillating edge leavingvariable degrees of absolute or relative scotoma in itswake. In other cases, scotoma without positive phenomenamay occur; this is often perceived as beingof acute onset but, on scrutiny, usually enlargesgradually. Next in frequency are sensory disturbancesin the form of pins and needles movingslowly from the point of origin and affecting agreater or smaller part of one side of the body andface. Numbness may occur in its wake, but numbnessmay also be the only symptom. Less frequentare speech disturbances, usually dysphasic but oftenhard to categorise. If the aura includes motor weakness,code as 1.2.4 Familial hemiplegic migraine or 1.2.5Sporadic hemiplegic migraine.Symptoms usually follow one another in successionbeginning with visual, then sensory symptomsand dysphasia, but the reverse and other orders havebeen noted. Patients often find it hard to describetheir symptoms in which case they should beinstructed in how to time and record them. Aftersuch prospective observation the clinical pictureoften becomes clearer. Common mistakes are incorrectreports of lateralisation of headache, of suddenonset when it is gradual and of monocular visualdisturbances when they are homonymous, as wellas incorrect duration of aura and mistaking sensoryloss for weakness. After an initial consultation, useof an aura diary may clarify the diagnosis.1.2.2 Typical aura with non-migraine headacheDiagnosing headache type description:Typical aura consisting of visual and/or sensoryand/or speech symptoms. Gradual development,duration no longer than one hour, a mix of positiveand negative features and complete reversibilitycharacterise the aura which is associated with aheadache that does not fulfil criteria for 1.1 Migrainewithout aura.Diagnosing headache type diagnostic criteria:A. At least 2 attacks fulfilling criteria BDB. Aura consisting of at least one of the following,but no motor weakness:1. fully reversible visual symptoms includingpositive features (eg, flickering lights, spotsor lines) and/or negative features (ie, loss ofvision)2. fully reversible sensory symptoms includingpositive features (ie, pins and needles) and/ornegative features (ie, numbness)3. fully reversible dysphasic speech disturbanceC. At least two of the following:1. homonymous visual symptoms1 and/or unilateralsensory symptoms2. at least one aura symptom develops graduallyover ≥5 minutes and/or different aura symptomsoccur in succession over ≥5 minutes3. each symptom lasts ≥5 and £60 minutesD. Headache that does not fulfil criteria BD for 1.1Migraine without aura begins during the aura orfollows aura within 60 minutesE. Not attributed to another disorder2Notes:1. Additional loss or blurring of central vision mayoccur.2. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type (IHS 2003)28 ICHD-IIDiagnosing headache type comment:In the absence of headache fulfilling criteria for 1.1Migraine without aura, precise diagnosis of aura andits distinction from mimics that may signal seriousdisease (eg, transient ischaemic attack) become muchmore important.1.2.3 Typical aura without headacheDiagnosing headache type description:Typical aura consisting of visual and/or sensorysymptoms with or without speech symptoms.Gradual development, duration no longer than onehour, a mix of positive and negative features andcomplete reversibility characterise the aura which isnot associated with headache.Diagnosing headache type diagnostic criteria:A. At least 2 attacks fulfilling criteria BDB. Aura consisting of at least one of the following,with or without speech disturbance but no motorweakness:1. fully reversible visual symptoms includingpositive features (eg, flickering lights, spotsor lines) and/or negative features (ie, loss ofvision)2. fully reversible sensory symptoms includingpositive features (ie, pins and needles) and/ornegative features (ie, numbness)C. At least two of the following:1. homonymous visual symptoms1 and/or unilateralsensory symptoms2. at least one aura symptom develops graduallyover ≥5 minutes and/or different aura symptomsoccur in succession over ≥5 minutes3. each symptom lasts ≥5 and £60 minutesD. Headache does not occur during aura nor followaura within 60 minutesE. Not attributed to another disorder2Notes:1. Additional loss or blurring of central vision mayoccur.2. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type comments:In some patients a typical aura is always followed bymigraine headache, but many patients have, in addition,attacks with aura followed by non-migraineheadache or even without headache. A smallnumber of patients have 1.2.3 Typical aura withoutheadache exclusively. More commonly, as patientswith 1.2.1 Typical aura with migraine headache becomeolder, their headache may lose migraine characteristicsor disappear completely even though auras continue.Some individuals, primarily males, have 1.2.3Typical aura without headache from onset.In the absence of headache fulfilling criteria for 1.1Migraine without aura, precise diagnosis of aura andits distinction from mimics that may signal seriousdisease (eg, transient ischaemic attack) become muchmore important. This distinction may require investigation.Especially when aura begins after age 40,when negative features (eg, hemianopia) are predominant,or when aura is prolonged or very short,other causes should be ruled out.1.2.4 Familial hemiplegic migraine (FHM)Diagnosing headache type description:Migraine with aura including motor weakness andat least one first- or second-degree relative hasmigraine aura including motor weakness.Diagnosing headache type diagnostic criteria:A. At least 2 attacks fulfilling criteria B and CB. Aura consisting of fully reversible motor weaknessand at least one of the following:1. fully reversible visual symptoms includingpositive features (eg, flickering lights, spotsor lines) and/or negative features (ie, loss ofvision)2. fully reversible sensory symptoms includingpositive features (ie, pins and needles) and/ornegative features (ie, numbness)3. fully reversible dysphasic speech disturbanceC. At least two of the following:1. at least one aura symptom develops graduallyover ≥5 minutes and/or different aura symptomsoccur in succession over ≥5 minutes2. each aura symptom lasts ≥5 minutes and <24hours3. headache fulfilling criteria BD for 1.1Migraine without aura begins during the auraor follows onset of aura within 60 minutesD. At least one first- or second-degree relative hashad attacks fulfilling these criteria AEE. Not attributed to another disorder1Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 29Note:1. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type comments:It may be difficult to distinguish weakness fromsensory loss.New genetic data have allowed a more precisedefinition of FHM than previously. Specific geneticsubtypes of 1.2.4 Familial hemiplegic migraine havebeen identified: in FHM1 there are mutations in theCACNA1A gene on chromosome 19, and in FHM2mutations occur in the ATP1A2 gene on chromosome1. If genetic testing is done, the genetic subtypeshould be specified parenthetically.It has been shown that FHM1 very often hasbasilar-type symptoms in addition to the typicalaura symptoms and that headache is virtuallyalways present. During FHM1 attacks, disturbancesof consciousness (sometimes including coma), fever,CSF pleocytosis and confusion can occur. FHM1attacks can be triggered by (mild) head trauma. Inapproximately 50% of FHM1 families, chronic progressivecerebellar ataxia occurs independently ofthe migraine attacks.FHM is very often mistaken for epilepsy, and(unsuccessfully) treated as such.1.2.5 Sporadic hemiplegic migraineDiagnosing headache type description:Migraine with aura including motor weakness butno first- or second-degree relative has aura includingmotor weakness.Diagnosing headache type diagnostic criteria:A. At least 2 attacks fulfilling criteria B and CB. Aura consisting of fully reversible motor weaknessand at least one of the following:1. fully reversible visual symptoms includingpositive features (eg, flickering lights, spotsor lines) and/or negative features (ie, loss ofvision)2. fully reversible sensory symptoms includingpositive features (ie, pins and needles) and/ornegative features (ie, numbness)3. fully reversible dysphasic speech disturbanceC. At least two of the following:1. at least one aura symptom develops graduallyover ≥5 minutes and/or different aura symptomsoccur in succession over ≥5 minutes2. each aura symptom lasts ≥5 minutes and <24hours3. headache fulfilling criteria BD for 1.1Migraine without aura begins during the auraor follows onset of aura within 60 minutesD. No first- or second-degree relative has attacks fulfillingthese criteria AEE. Not attributed to another disorder1Note:1. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type comments:Epidemiological studies have shown that sporadiccases occur with approximately the same prevalenceas familial cases. The attacks have the same clinicalcharacteristics as those in 1.2.4 Familial hemiplegicmigraine.Sporadic cases always require neuroimaging andother tests to rule out other cause. A lumbar punctureis also necessary to rule out pseudomigrainewith temporary neurological symptoms and lymphocyticpleocytosis. This condition is more prevalentin males and often associated with transienthemiparesis and aphasia.1.2.6 Basilar-type migraineDiagnosing headache type previously used terms:Basilar artery migraine, basilar migraineDiagnosing headache type description:Migraine with aura symptoms clearly originatingfrom the brainstem and/or from both hemispheressimultaneously affected, but no motor weakness.Diagnosing headache type diagnostic criteria:A. At least 2 attacks fulfilling criteria BDB. Aura consisting of at least two of the followingfully reversible symptoms, but no motorweakness:1. dysarthria2. vertigoDiagnosing headache type (IHS 2003)30 ICHD-II3. tinnitus4. hypacusia5. diplopia6. visual symptoms simultaneously in both temporaland nasal fields of both eyes7. ataxia8. decreased level of consciousness9. simultaneously bilateral paraesthesiasC. At least one of the following:1. at least one aura symptom develops graduallyover ≥5 minutes and/or different aura symptomsoccur in succession over ≥5 minutes2. each aura symptom lasts ≥5 and £60 minutesD. Headache fulfilling criteria BD for 1.1 Migrainewithout aura begins during the aura or followsaura within 60 minutesE. Not attributed to another disorder1Note:1. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type comments:Basilar-type attacks are mostly seen in young adults.Many patients who have basilar-type attacks alsoreport attacks with typical aura (code for bothdisorders).If motor weakness is present, code as 1.2.4 Familialhemiplegic migraine or 1.2.5 Sporadic hemiplegicmigraine. Patients with 1.2.4 Familial hemiplegicmigraine have basilar-type symptoms in 60% ofcases. Therefore, 1.2.6 Basilar-type migraine should bediagnosed only when no motor weakness occurs.Many of the symptoms listed under criterion B aresubject to misinterpretation as they may occur withanxiety and hyperventilation.Originally the terms basilar artery migraine orbasilar migraine were used but, since involvement ofthe basilar artery territory is uncertain (ie, the disturbancemay be bihemispheric), the term basilartypemigraine is preferred.1.3 Childhood periodic syndromes that are commonlyprecursors of migraine1.3.1 Cyclical vomitingDiagnosing headache type description:Recurrent episodic attacks, usually stereotypicalin the individual patient, of vomiting and intensenausea. Attacks are associated with pallor andlethargy. There is complete resolution of symptomsbetween attacks.Diagnosing headache type diagnostic criteria:A. At least 5 attacks fulfilling criteria B and CB. Episodic attacks, stereotypical in the individualpatient, of intense nausea and vomiting lastingfrom 1 hour to 5 daysC. Vomiting during attacks occurs at least 4times/hour for at least 1 hourD. Symptom-free between attacksE. Not attributed to another disorder1Note:1. In particular, history and physical examination donot show signs of gastrointestinal disease.Diagnosing headache type comment:Cyclical vomiting is a self-limiting episodic conditionof childhood, with periods of complete normalitybetween episodes. This disorder was notincluded as a childhood periodic syndrome in thefirst edition of The International Classification ofHeadache Disorders. The clinical features of thissyndrome resemble those found in associationwith migraine headaches, and multiple threads ofresearch over the last years have suggested thatcyclical vomiting is a condition related to migraine.1.3.2 Abdominal migraineDiagnosing headache type description:An idiopathic recurrent disorder seen mainly in childrenand characterised by episodic midline abdominalpain manifesting in attacks lasting 172 hourswith normality between episodes. The pain is ofmoderate to severe intensity and associated withvasomotor symptoms, nausea and vomiting.Diagnosing headache type diagnostic criteria:A. At least 5 attacks fulfilling criteria BDB. Attacks of abdominal pain lasting 172 hours(untreated or unsuccessfully treated)C. Abdominal pain has all of the followingcharacteristics:Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 311. midline location, periumbilical or poorlylocalised2. dull or just sore quality3. moderate or severe intensityD. During abdominal pain at least 2 of thefollowing:1. anorexia2. nausea3. vomiting4. pallorE. Not attributed to another disorder1Note:1. In particular, history and physical examination donot show signs of gastrointestinal or renal diseaseor such disease has been ruled out by appropriateinvestigations.Diagnosing headache type comments:Pain is severe enough to interfere with normal dailyactivities.Children may find it difficult to distinguishanorexia from nausea. The pallor is often accompaniedby dark shadows under the eyes. In a fewpatients flushing is the predominant vasomotorphenomenon.Most children with abdominal migraine willdevelop migraine headache later in life.1.3.3 Benign paroxysmal vertigo of childhoodDiagnosing headache type description:This probably heterogeneous disorder is characterisedby recurrent brief episodic attacks of vertigooccurring without warning and resolving spontaneouslyin otherwise healthy children.Diagnosing headache type diagnostic criteria:A. At least 5 attacks fulfilling criterion BB. Multiple episodes of severe vertigo1, occurringwithout warning and resolving spontaneouslyafter minutes to hoursC. Normal neurological examination and audiometricand vestibular functions between attacksD. Normal electroencephalogramNote:1. Often associated with nystagmus or vomiting;unilateral throbbing headache may occur in someattacks.1.4 Retinal migraineDiagnosing headache type description:Repeated attacks of monocular visual disturbance,including scintillations, scotomata or blindness,associated with migraine headache.Diagnosing headache type diagnostic criteria:A. At least 2 attacks fulfilling criteria B and CB. Fully reversible monocular positive and/ornegative visual phenomena (eg, scintillations,scotomata or blindness) confirmed by examinationduring an attack or (after proper instruction)by the patients drawing of a monocular fielddefect during an attackC. Headache fulfilling criteria BD for 1.1 Migrainewithout aura begins during the visual symptomsor follows them within 60 minutesD. Normal ophthalmological examination betweenattacksE. Not attributed to another disorder1Note:1. Appropriate investigations exclude other causesof transient monocular blindness.Diagnosing headache type comment:Some patients who complain of monocular visualdisturbance in fact have hemianopia. Some caseswithout headache have been reported, but theirmigrainous nature cannot be ascertained. Othercauses of transient monocular blindness (amaurosisfugax), such as optic neuropathy or carotid dissection,must be excluded.1.5 Complications of migraineDiagnosing headache type comment:Code separately for both the antecedent migrainesubtype and for the complication.1.5.1 Chronic migraineDiagnosing headache type description:Migraine headache occurring on 15 or more days permonth for more than 3 months in the absence ofmedication overuse.Diagnosing headache type diagnostic criteria:A. Headache fulfilling criteria C and D for 1.1Migraine without aura on ≥15 days/month for >3monthsB. Not attributed to another disorder1;2Diagnosing headache type (IHS 2003)32 ICHD-IINotes:1. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but headachedoes not occur for the first time in close temporalrelation to the disorder.2. When medication overuse is present and fulfilscriterion B for any of the subforms of 8.2 Medication-overuse headache, it is uncertain whether criterionB for 1.5.1 Chronic migraine is fulfilled until 2months after medication has been withdrawnwithout improvement (see diagnosing headache type comments).Diagnosing headache type comments:Most cases of chronic migraine start as 1.1 Migrainewithout aura. Therefore, chronicity may be regardedas a complication of episodic migraine.As chronicity develops, headache tends to lose itsattack-wise (episodic) presentation although it hasnot been clearly demonstrated that this is always so.When medication overuse is present (ie, fulfillingcriterion B for any of the subforms of 8.2 Medicationoveruseheadache), this is the most likely cause ofchronic symptoms. Therefore, the default rule is tocode such patients according to the antecedentmigraine subtype (usually 1.1 Migraine without aura)plus 1.6.5 Probable chronic migraine plus 8.2.7 Probablemedication-overuse headache. When these criteria arestill fulfilled 2 months after medication overuse hasceased, 1.5.1 Chronic migraine plus the antecedentmigraine subtype should be diagnosed, and 8.2.7Probable medication-overuse headache discarded. If atany time sooner they are no longer fulfilled, becauseimprovement has occurred, code for 8.2 Medicationoveruseheadache plus the antecedent migrainesubtype and discard 1.6.5 Probable chronic migraine.These criteria require further study.1.5.2 Status migrainosusDiagnosing headache type description:A debilitating migraine attack lasting for more than72 hours.Diagnosing headache type diagnostic criteria:A. The present attack in a patient with 1.1 Migrainewithout aura is typical of previous attacks exceptfor its durationB. Headache has both of the following features:1. unremitting for >72 hours2. severe intensityC. Not attributed to another disorderDiagnosing headache type comment:Interruption during sleep is disregarded. Shortlastingrelief due to medication is also disregarded.Status may often be caused by medication overuseand should be coded accordingly. Non-debilitatingattacks lasting >72 hours but otherwise meetingthese criteria are coded as 1.6.1 Probable migrainewithout aura.1.5.3 Persistent aura without infarctionDiagnosing headache type description:Aura symptoms persisting for more than 1 weekwithout radiographic evidence of infarction.Diagnosing headache type diagnostic criteria:A. The present attack in a patient with 1.2 Migrainewith aura is typical of previous attacks except thatone or more aura symptoms persists for >1 weekB. Not attributed to another disorderDiagnosing headache type comments:Persisting aura symptoms are rare but well documented.They are often bilateral and may last formonths or years. Reliably effective treatment is notknown though acetazolamide and valproic acidhave helped in a few cases.Exclude posterior leukoencephalopathy by diffusionMRI among other things. Exclude 1.5.4 Migrainousinfarction by MRI.1.5.4 Migrainous infarctionDiagnosing headache type description:One or more migrainous aura symptoms associatedwith an ischaemic brain lesion in appropriate territorydemonstrated by neuroimaging.Diagnosing headache type diagnostic criteria:A. The present attack in a patient with 1.2 Migrainewith aura is typical of previous attacks except thatone or more aura symptoms persists for >60minutesB. Neuroimaging demonstrates ischaemic infarctionin a relevant areaC. Not attributed to another disorderDiagnosing headache type comments:Ischaemic stroke in a migraine sufferer may be categorisedas cerebral infarction of other cause coexistingwith migraine, cerebral infarction of other causepresenting with symptoms resembling migraineDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 33with aura, or cerebral infarction occurring during thecourse of a typical migraine with aura attack. Onlythe last fulfils criteria for 1.5.4 Migrainous infarction.Increased risk for stroke in migraine patients hasbeen demonstrated in women under age 45 inseveral studies. Evidence for an association betweenmigraine and stroke in older women and in men isinconsistent.1.5.5 Migraine-triggered seizureDiagnosing headache type description:A seizure triggered by a migraine aura.Diagnosing headache type diagnostic criteria:A. Migraine fulfilling criteria for 1.2 Migraine withauraB. Aseizure fulfilling diagnostic criteria for one typeof epileptic attack occurs during or within 1 hourafter a migraine auraDiagnosing headache type comment:Migraine and epilepsy are prototypical examplesof paroxysmal brain disorders. While migraine-likeheadaches are quite frequently seen in the postictalperiod, sometimes a seizure occurs during or followinga migraine attack. This phenomenon, sometimesreferred to as migralepsy, has been described inpatients with migraine with aura.1.6 Probable migraineDiagnosing headache type previously used terms:Migrainous disorderCoded elsewhere:Migraine-like headache secondary to another disorder(symptomatic migraine) is coded according to thatdisorder.Diagnosing headache type description:Attacks and/or headache missing one of the featuresneeded to fulfil all criteria for a disorder coded above(1.6.3 Probable childhood periodic syndromes that arecommonly precursors of migraine and 1.6.4 Probableretinal migraine are not currently recognised).1.6.1 Probable migraine without auraDiagnosing headache type diagnostic criteria:A. Attacks fulfilling all but one of criteria AD for1.1 Migraine without auraB. Not attributed to another disorderDiagnosing headache type comment:Do not code as 1.6.1 Probable migraine without auraif the patient fulfils the criteria for 1.5.1 Chronicmigraine or 1.5.2 Status migrainosus.1.6.2 Probable migraine with auraDiagnosing headache type diagnostic criteria:A. Attacks fulfilling all but one of criteria AD for1.2 Migraine with aura or any of its subformsB. Not attributed to another disorder1.6.5 Probable chronic migraineDiagnosing headache type diagnostic criteria:A. Headache fulfilling criteria C and D for 1.1Migraine without aura on ≥15 days/month for >3monthsB. Not attributed to another disorder1 but there is,or has been within the last 2 months, medicationoveruse fulfilling criterion B for any of the subformsof 8.2 Medication-overuse headacheNote:1. History and physical and neurological examinationsdo not suggest any of the disorders listedin groups 512 (other than 8.2 Medication-overuseheadache), or history and/or physical and/or neurologicalexaminations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but headache does notoccur for the first time in close temporal relationto the disorder.Aggravating factorsMigraine may be aggravated by a number of factors.That is, in a person who already meets criteria formigraine, particular factors may be associated witha relatively long-term (usually weeks to months)increase in the severity or frequency of attacks.Examples of commonly-reported aggravatingfactors include: psychosocial stress, frequent intakeof alcoholic beverages, other environmental factors.Trigger factors (precipitating factors)Trigger factors increase the probability of a migraineattack in the short term (usually <48 hours) in aperson with migraine. 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Cephalalgia 1996; 16:4315.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 372. Tension-type headache (TTH)2.1 Infrequent episodic tension-type headache2.1.1 Infrequent episodic tension-typeheadache associated with pericranialtenderness2.1.2 Infrequent episodic tension-typeheadache not associated with pericranialtenderness2.2 Frequent episodic tension-type headache2.2.1 Frequent episodic tension-type headacheassociated with pericranial tenderness2.2.2 Frequent episodic tension-type headachenot associated with pericranial tenderness2.3 Chronic tension-type headache2.3.1 Chronic tension-type headache associatedwith pericranial tenderness2.3.2 Chronic tension-type headache notassociated with pericranial tenderness2.4 Probable tension-type headache2.4.1 Probable infrequent episodic tension-typeheadache2.4.2 Probable frequent episodic tension-typeheadache2.4.3 Probable chronic tension-type headacheDiagnosing headache type previously used terms:Tension headache, muscle contraction headache,psychomyogenic headache, stress headache, ordinaryheadache, essential headache, idiopathicheadache and psychogenic headacheCoded elsewhere:Tension-type-like headache attributed to anotherdisorder is coded to that disorder.General commentPrimary or secondary headache or both?When a headache with tension-type characteristicsoccurs for the first time in close temporal relation toanother disorder that is a known cause of headache,it is coded according to the causative disorder as asecondary headache. When pre-existing tensiontypeheadache is made worse in close temporal relationto another disorder that is a known cause ofheadache, there are two possibilities, and judgmentis required. The patient can either be given only thetension-type headache diagnosis or be given boththe tension-type headache diagnosis and a secondaryheadache diagnosis according to the other disorder.Factors that support adding the latterdiagnosis are: a very close temporal relation to thedisorder, a marked worsening of the tension-typeheadache, very good evidence that the disordercan cause or aggravate tension-type headache and,finally, improvement or resolution of tension-typeheadache after relief from the disorder.IntroductionThis is the most common type of primary headache:its lifetime prevalence in the general populationranges in different studies from 30 to 78%. At thesame time, it is the least studied of the primaryheadache disorders, despite the fact that it has thehighest socio-economic impact.Whilst this type of headache was previously consideredto be primarily psychogenic, a number ofstudies have appeared after the first edition of TheInternational Classification of Headache Disorders thatstrongly suggest a neurobiological basis, at least forthe more severe subtypes of tension-type headache.The division into episodic and chronic subtypes thatwas introduced in the first edition of the classificationhas proved extremely useful. The chronicsubtype is a serious disease causing greatlydecreased quality of life and high disability. In thepresent edition we have decided to subdivideepisodic tension-type headache further, into an infrequentsubtype with headache episodes less than onceper month and a frequent subtype. The infrequentsubtype has very little impact on the individual anddoes not deserve much attention from the medicalprofession. However, frequent sufferers canencounter considerable disability that sometimeswarrants expensive drugs and prophylactic medication.The chronic subtype is of course always associatedwith disability and high personal andsocio-economic costs.The first edition arbitrarily separated patientswith and without disorder of the pericranialmuscles. This has proved to be a valid subdivisionbut the only really useful distinguishing feature istenderness on manual palpation and not, as suggestedin the first edition, evidence from surfaceEMG or pressure algometry. Therefore, we now useonly manual palpation, preferably as pressurecontrolledpalpation, to subdivide all three subtypesof tension-type headache.The exact mechanisms of tension-type headacheare not known. Peripheral pain mechanisms aremost likely to play a role in 2.1 Infrequent episodictension-type headache and 2.2 Frequent episodic tensiontypeheadache whereas central pain mechanisms playa more important role in 2.3 Chronic tension-typeheadache. The classification subcommittee encour-Diagnosing headache type (IHS 2003)38 ICHD-IIages further research into the pathophysiologicalmechanisms and treatment of tension-typeheadache.There are some reasons to believe that, with thediagnostic criteria set out in the first edition, patientscoded for episodic tension-type headache includedsome who had a mild form of migraine without auraand patients coded for chronic tension-typeheadache included some who had chronic migraine.Clinical experience favours this suspicion, especiallyin patients who also have migraine attacks, andsome patients may display pathophysiological featurestypical of migraine (Schoenen et al., 1987).Within the classification subcommittee there was anattempt to tighten the diagnostic criteria for tensiontypeheadache for the second edition, with thehope to exclude migraine patients whose headachephenotypically resembles tension-type headache.However, this would have compromised the sensitivityof the criteria and there was no evidence toshow the beneficial effects of such a change. Thereforea consensus was not reached, but a proposal fornew, stricter diagnostic criteria is published underA2 Tension-type headache in the appendix. The classificationsubcommittee recommends comparisonsbetween patients diagnosed according to the explicitcriteria and others diagnosed according to theappendix criteria. This pertains not only to theclinical features but also to pathophysiologicalmechanisms and response to treatments.2.1 Infrequent episodic tension-type headacheDiagnosing headache type description:Infrequent episodes of headache lasting minutes todays. The pain is typically bilateral, pressing ortightening in quality and of mild to moderate intensity,and it does not worsen with routine physicalactivity. There is no nausea but photophobia orphonophobia may be present.Diagnosing headache type diagnostic criteria:A. At least 10 episodes occurring on <1 day permonth on average (<12 days per year) and fulfillingcriteria BDB. Headache lasting from 30 minutes to 7 daysC. Headache has at least two of the followingcharacteristics:1. bilateral location2. pressing/tightening (non-pulsating) quality3. mild or moderate intensity4. not aggravated by routine physical activitysuch as walking or climbing stairsD. Both of the following:1. no nausea or vomiting (anorexia may occur)2. no more than one of photophobia orphonophobiaE. Not attributed to another disorder1Note:1. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but headachedoes not occur for the first time in close temporalrelation to the disorder.2.1.1 Infrequent episodic tension-type headacheassociated with pericranial tendernessDiagnosing headache type diagnostic criteria:A. Episodes fulfilling criteria AE for 2.1 Infrequentepisodic tension-type headacheB. Increased pericranial tenderness on manualpalpation2.1.2 Infrequent episodic tension-type headachenot associated with pericranial tendernessDiagnosing headache type diagnostic criteria:A. Episodes fulfilling criteria AE for 2.1 Infrequentepisodic tension-type headacheB. No increased pericranial tendernessDiagnosing headache type comments:Increased pericranial tenderness recorded bymanual palpation is the most significant abnormalfinding in patients with tension-type headache. Thetenderness increases with the intensity and frequencyof headache and is further increased duringactual headache. The diagnostic value of EMG andpressure algometry is limited and these recordingsare therefore omitted from the second edition. Pericranialtenderness is easily recorded by manualpalpation by small rotating movements and a firmpressure (preferably aided by use of a palpometer)with the second and third finger on the frontal, temporal,masseter, pterygoid, sternocleidomastoid,splenius and trapezius muscles. A local tendernessscore from 03 on each muscle can be summated toyield a total tenderness score for each individual. Ithas been demonstrated that, using a pressure sensitivedevice that allows palpation with a controlledpressure, this clinical examination becomes morevalid and reproducible. However, such equipment isnot generally available to clinicians and it is advisedDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 39that clinicians simply perform the manual palpationas a traditional clinical examination.Palpation is a useful guide for the treatment strategy.It also adds value and credibility to the explanationsgiven to the patient.2.2 Frequent episodic tension-type headacheDiagnosing headache type description:Frequent episodes of headache lasting minutes todays. The pain is typically bilateral, pressing ortightening in quality and of mild to moderate intensity,and it does not worsen with routine physicalactivity. There is no nausea but photophobia orphonophobia may be present.Diagnostic criteriaA. At least 10 episodes occurring on ≥1 but <15 daysper month for at least 3 months (≥12 and <180days per year) and fulfilling criteria BDB. Headache lasting from 30 minutes to 7 daysC. Headache has at least two of the followingcharacteristics:1. bilateral location2. pressing/tightening (non-pulsating) quality3. mild or moderate intensity4. not aggravated by routine physical activitysuch as walking or climbing stairsD. Both of the following:1. no nausea or vomiting (anorexia may occur)2. no more than one of photophobia orphonophobiaE. Not attributed to another disorder1Note:1. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but headachedoes not occur for the first time in close temporalrelation to the disorder.Diagnosing headache type comment:Frequent tension-type headache often coexists withmigraine without aura. Coexisting tension-typeheadache in migraineurs should preferably be identifiedby a diagnostic headache diary. The treatmentof migraine differs considerably from that of tensiontypeheadache and it is important to educate patientsto differentiate between these types of headaches inorder to select the right treatment and to preventmedication-overuse headache.2.2.1 Frequent episodic tension-type headacheassociated with pericranial tendernessDiagnosing headache type diagnostic criteria:A. Episodes fulfilling criteria AE for 2.2 Frequentepisodic tension-type headacheB. Increased pericranial tenderness on manualpalpation2.2.2 Frequent episodic tension-type headache notassociated with pericranial tendernessDiagnosing headache type diagnostic criteria:A. Episodes fulfilling criteria AE for 2.2 Frequentepisodic tension-type headacheB. No increased pericranial tenderness2.3 Chronic tension-type headacheCoded elsewhere:4.8 New daily-persistent headacheDiagnosing headache type description:A disorder evolving from episodic tension-typeheadache, with daily or very frequent episodes ofheadache lasting minutes to days. The pain is typicallybilateral, pressing or tightening in quality andof mild to moderate intensity, and it does not worsenwith routine physical activity. There may be mildnausea, photophobia or phonophobia.Diagnosing headache type diagnostic criteria:A. Headache occurring on ≥15 days per month onaverage for >3 months (≥180 days per year)1 andfulfilling criteria BDB. Headache lasts hours or may be continuousC. Headache has at least two of the followingcharacteristics:1. bilateral location2. pressing/tightening (non-pulsating) quality3. mild or moderate intensity4. not aggravated by routine physical activitysuch as walking or climbing stairsD. Both of the following:1. no more than one of photophobia, phonophobiaor mild nausea2. neither moderate or severe nausea norvomitingE. Not attributed to another disorder2;3Notes:1. 2.3 Chronic tension-type headache evolves over timefrom episodic tension-type headache; when thesecriteria AE are fulfilled by headache that, unam-Diagnosing headache type (IHS 2003)40 ICHD-IIbiguously, is daily and unremitting within 3 daysof its first onset, code as 4.8 New daily-persistentheadache. When the manner of onset is not rememberedor is otherwise uncertain, code as 2.3Chronic tension-type headache.2. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but headachedoes not occur for the first time in close temporalrelation to the disorder.3. When medication overuse is present and fulfilscriterion B for any of the subforms of 8.2 Medication-overuse headache, it is uncertain whether thiscriterion E is fulfilled until 2 months after medicationhas been withdrawn without improvement(see diagnosing headache type comments).Diagnosing headache type comments:The introduction of 1.5.1 Chronic migraine into TheInternational Classification of Headache Disorders, 2ndedition, creates a problem in relation to the differentialdiagnosis between this and 2.3 Chronic tensiontypeheadache. Both diagnoses require headache(meeting the criteria for migraine or tension-typeheadache respectively) on at least 15 days a month.Therefore it is possible theoretically that a patientcan have both these diagnoses. A very small groupof patients have 15 or more headaches per monthfulfilling the diagnostic criteria for both 1.5.1 Chronicmigraine and 2.3 Chronic tension-type headache. This ispossible when two (and only two) of the four paincharacteristics are present and headaches are associatedwith mild nausea. In these rare cases, otherclinical evidence that is not part of the explicit diagnosticcriteria should be taken into account and theclinician should base thereon the best possible choiceof diagnosis. When it is uncertain how many attacksfulfil one or other set of criteria it is strongly recommendedto use a diagnostic headache diaryprospectively.In many uncertain cases there is overuse ofmedication. When this fulfils criterion B for any ofthe subforms of 8.2 Medication-overuse headache, thedefault rule is to code for 2.4.3 Probable chronictension-type headache plus 8.2.7 Probable medicationoveruseheadache. When these criteria are still fulfilled2 months after medication overuse has ceased, 2.3Chronic tension-type headache should be diagnosedand 8.2.7 Probable medication-overuse headache discarded.If at any time sooner they are no longerfulfilled, because improvement has occurred, 8.2Medication-overuse headache should be diagnosed and2.4.3 Probable chronic tension-type headache discarded.It should be remembered that some patients withchronic tension-type headache develop migrainelikefeatures if they have severe pain and, conversely,some migraine patients develop increasingly frequenttension-type-like interval headaches, thenature of which remains unclear.2.3.1 Chronic tension-type headache associatedwith pericranial tendernessDiagnosing headache type diagnostic criteria:A. Headache fulfilling criteria AE for 2.3 Chronictension-type headacheB. Increased pericranial tenderness on manualpalpation2.3.2 Chronic tension-type headache not associatedwith pericranial tendernessDiagnosing headache type diagnostic criteria:A. Headache fulfilling criteria AE for 2.3 Chronictension-type headacheB. No increased pericranial tenderness2.4 Probable tension-type headacheDiagnosing headache type comment:Patients meeting one of these sets of criteria may alsomeet the criteria for one of the subforms of 1.6 Probablemigraine. In such cases, all other available informationshould be used to decide which of thealternatives is the more likely.2.4.1 Probable infrequent episodic tension-typeheadacheDiagnosing headache type diagnostic criteria:A. Episodes fulfilling all but one of criteria AD for2.1 Infrequent episodic tension-type headacheB. Episodes do not fulfil criteria for 1.1 Migrainewithout auraC. Not attributed to another disorder2.4.2 Probable frequent episodic tension-typeheadacheDiagnosing headache type diagnostic criteria:A. Episodes fulfilling all but one of criteria AD for2.2 Frequent episodic tension-type headacheB. Episodes do not fulfil criteria for 1.1 Migrainewithout auraC. Not attributed to another disorderDiagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 412.4.3 Probable chronic tension-type headacheDiagnosing headache type diagnostic criteria:A. Headache occurring on ≥15 days per month onaverage for >3 months (≥180 days per year) andfulfilling criteria BDB. Headache lasts hours or may be continuousC. Headache has at least two of the followingcharacteristics:1. bilateral location2. pressing/tightening (non-pulsating) quality3. mild or moderate intensity4. not aggravated by routine physical activitysuch as walking or climbing stairsD. Both of the following:1. no more than one of photophobia, phonophobiaor mild nausea2. neither moderate or severe nausea norvomitingE. 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Cluster headache and other trigeminalautonomic cephalalgias3.1 Cluster headache3.1.1 Episodic cluster headache3.1.2 Chronic cluster headache3.2 Paroxysmal hemicrania3.2.1 Episodic paroxysmal hemicrania3.2.2 Chronic paroxysmal hemicrania (CPH)3.3 Short-lasting unilateral neuralgiform headacheattacks with conjunctival injection and tearing(SUNCT)3.4 Probable trigeminal autonomic cephalalgia3.4.1 Probable cluster headache3.4.2 Probable paroxysmal hemicrania3.4.3 Probable SUNCTCoded elsewhere:4.7 Hemicrania continuaGeneral commentPrimary or secondary headache or both?When a headache with the characteristics of atrigeminal autonomic cephalalgia (TAC) occurs forthe first time in close temporal relation to anotherdisorder that is a known cause of headache, it iscoded according to the causative disorder as a secondaryheadache. When a pre-existing TAC is madeworse in close temporal relation to another disorderthat is a known cause of headache, there are two possibilities,and judgment is required. The patient caneither be given only the TAC diagnosis or be givenboth the TAC diagnosis and a secondary headachediagnosis according to the other disorder. Factorsthat support adding the latter diagnosis are: a veryclose temporal relation to the disorder, a markedworsening of the TAC, very good evidence that thedisorder can cause or aggravate the TAC and, finally,improvement or resolution of the TAC after relieffrom the disorder.IntroductionThe trigeminal autonomic cephalalgias share theclinical features of headache and prominent cranialparasympathetic autonomic features. Experimentaland human functional imaging suggests that thesesyndromes activate a normal human trigeminalparasympatheticreflex with clinical signs of cranialsympathetic dysfunction being secondary.Hemicrania continua, whose cranial autonomic featuresare less constant, is to be found under 4. Otherprimary headaches.3.1 Cluster headacheDiagnosing headache type previously used terms:Ciliary neuralgia, erythro-melalgia of the head, erythroprosopalgiaof Bing, hemicrania angioparalytica,hemicrania neuralgiformis chronica,histaminic cephalalgia, Hortons headache, Harris-Hortons disease, migrainous neuralgia (of Harris),petrosal neuralgia (of Gardner).Coded elsewhere:Symptomatic cluster headache is coded to the underlyingcausative disorder.Diagnosing headache type description:Attacks of severe, strictly unilateral pain which isorbital, supraorbital, temporal or in any combinationof these sites, lasting 15180 minutes and occurringfrom once every other day to 8 times a day. Theattacks are associated with one or more of the following,all of which are ipsilateral: conjunctivalinjection, lacrimation, nasal congestion, rhinorrhoea,forehead and facial sweating, miosis, ptosis, eyelidoedema. Most patients are restless or agitated duringan attack.Diagnosing headache type diagnostic criteria:A. At least 5 attacks fulfilling criteria BDB. Severe or very severe unilateral orbital, supraorbitaland/or temporal pain lasting 15180minutes if untreated1C. Headache is accompanied by at least one of thefollowing:1. ipsilateral conjunctival injection and/orlacrimation2. ipsilateral nasal congestion and/or rhinorrhoea3. ipsilateral eyelid oedema4. ipsilateral forehead and facial sweating5. ipsilateral miosis and/or ptosis6. a sense of restlessness or agitationD. Attacks have a frequency from one every otherday to 8 per day2E. Not attributed to another disorder3Notes:1. During part (but less than half) of the time-courseof cluster headache, attacks may be less severeand/or of shorter or longer duration.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 452. During part (but less than half) of the time-courseof cluster headache, attacks may be less frequent.3. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type comments:Acute attacks involve activation of the posteriorhypothalamic grey matter. Cluster headache maybe inherited (autosomal dominant) in about 5% ofcases.Attacks usually occur in series (cluster periods)lasting for weeks or months separated by remissionperiods usually lasting months or years. However,about 1015% of patients have chronic symptomswithout remissions.In a large series with good follow-up, 27% ofpatients had only a single cluster period. Theseshould be coded as 3.1 Cluster headache.During a cluster period, and in the chronicsubtype, attacks occur regularly and may be provokedby alcohol, histamine or nitroglycerine. Painis maximal orbitally, supraorbitally, temporally or inany combination of these sites, but may spread toother regions of the head. Pain almost invariablyrecurs on the same side during an individual clusterperiod. During the worst attacks, the intensity ofpain is excruciating. Patients are usually unable tolie down and characteristically pace the floor.Age at onset is usually 2040 years. For unknownreasons prevalence is 34 times higher in men thanin women.Cluster headache with coexistent trigeminalneuralgia (cluster-tic syndrome):Some patients have been described who have both3.1 Cluster headache and 13.1 Trigeminal neuralgia.They should receive both diagnoses. The importanceof this observation is that both conditions must betreated for the patient to be headache free.3.1.1 Episodic cluster headacheDiagnosing headache type description:Cluster headache attacks occurring in periodslasting 7 days to 1 year separated by pain-freeperiods lasting 1 month or longer.Diagnosing headache type diagnostic criteria:A. Attacks fulfilling criteria AE for 3.1 ClusterheadacheB. At least two cluster periods lasting 7365 days1and separated by pain-free remission periods of≥1 monthNote:1. Cluster periods usually last between 2 weeks and3 months.Diagnosing headache type comment:The duration of the remission period has beenincreased in this second edition to a minimum of 1month.3.1.2 Chronic cluster headacheDiagnosing headache type description:Cluster headache attacks occurring for more than 1year without remission or with remissions lastingless than 1 month.Diagnosing headache type diagnostic criteria:A. Attacks fulfilling criteria AE for 3.1 ClusterheadacheB. Attacks recur over >1 year without remissionperiods or with remission periods lasting <1monthDiagnosing headache type comments:Chronic cluster headache may arise de novo (previouslyreferred to as primary chronic cluster headache)or evolve from the episodic subtype (previouslyreferred to as secondary chronic cluster headache). Somepatients may switch from chronic to episodic clusterheadache.3.2 Paroxysmal hemicraniaDiagnosing headache type description:Attacks with similar characteristics of pain and associatedsymptoms and signs to those of clusterheadache, but they are shorter-lasting, more frequent,occur more commonly in females andrespond absolutely to indomethacin.Diagnosing headache type diagnostic criteria:A. At least 20 attacks fulfilling criteria BDB. Attacks of severe unilateral orbital, supraorbitalor temporal pain lasting 230 minutesC. Headache is accompanied by at least one of thefollowing:Diagnosing headache type (IHS 2003)46 ICHD-II1. ipsilateral conjunctival injection and/orlacrimation2. ipsilateral nasal congestion and/or rhinorrhoea3. ipsilateral eyelid oedema4. ipsilateral forehead and facial sweating5. ipsilateral miosis and/or ptosisD. Attacks have a frequency above 5 per day formore than half of the time, although periods withlower frequency may occurE. Attacks are prevented completely by therapeuticdoses of indomethacin1F. Not attributed to another disorder2Notes:1. In order to rule out incomplete response,indomethacin should be used in a dose of ≥150mgdaily orally or rectally, or ≥100 mg by injection,but for maintenance smaller doses are oftensufficient.2. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type comments:There is no male predominance. Onset is usually inadulthood, although childhood cases are reported.In the first edition all paroxysmal hemicraniaswere referred to as chronic paroxysmal hemicrania. Sufficientclinical evidence for the episodic subtype hasaccumulated to separate it in a manner analogous tocluster headache.Paroxysmal hemicrania with coexistent trigeminalneuralgia (CPH-tic syndrome):Patients who fulfil criteria for both 3.2 Paroxysmalhemicrania and 13.1 Trigeminal neuralgia shouldreceive both diagnoses. The importance of thisobservation is that both conditions require treatment.The pathophysiological significance of theassociation is not yet clear.3.2.1 Episodic paroxysmal hemicraniaDiagnosing headache type description:Attacks of paroxysmal hemicrania occurring inperiods lasting 7 days to 1 year separated by painfreeperiods lasting 1 month or longer.Diagnosing headache type diagnostic criteria:A. Attacks fulfilling criteria AF for 3.2 ParoxysmalhemicraniaB. At least two attack periods lasting 7365 daysand separated by pain-free remission periods of≥1 month3.2.2 Chronic paroxysmal hemicrania (CPH)Diagnosing headache type description:Attacks of paroxysmal hemicrania occurring formore than 1 year without remission or with remissionslasting less than 1 month.Diagnosing headache type diagnostic criteria:A. Attacks fulfilling criteria AF for 3.2 ParoxysmalhemicraniaB. Attacks recur over >1 year without remissionperiods or with remission periods lasting <1month3.3 Short-lasting Unilateral Neuralgiform headacheattacks with Conjunctival injection and Tearing(SUNCT)Diagnosing headache type description:This syndrome is characterised by short-lastingattacks of unilateral pain that are much briefer thanthose seen in any other TAC and very often accompaniedby prominent lacrimation and redness of theipsilateral eye.Diagnosing headache type diagnostic criteria:A. At least 20 attacks fulfilling criteria BDB. Attacks of unilateral orbital, supraorbital or temporalstabbing or pulsating pain lasting 5240secondsC. Pain is accompanied by ipsilateral conjunctivalinjection and lacrimationD. Attacks occur with a frequency from 3 to 200 perdayE. Not attributed to another disorder1Note:1. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but attacks donot occur for the first time in close temporal relationto the disorder.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 47Diagnosing headache type comments:This syndrome was described after the publicationof the first edition of The International Classification ofHeadache Disorders and has become well recognisedin the last decade.Patients may be seen with only one of conjunctivalinjection or tearing, or other cranial autonomicsymptoms such as nasal congestion, rhinorrhoea oreyelid oedema may be seen. 3.3 SUNCT may be asubform of A3.3 Short-lasting Unilateral Neuralgiformheadache attacks with cranial Autonomic symptoms(SUNA), described in the appendix.The literature suggests that the most commonmimics of 3.3 SUNCT are lesions in the posteriorfossa or involving the pituitary gland.SUNCT with coexistent trigeminal neuralgia:Patients have been described in whom there is anoverlap between 3.3 SUNCT and 13.1 Trigeminal neuralgia.Such patients should receive both diagnoses.This differentiation is clinically difficult.3.4 Probable trigeminal autonomic cephalalgiaDiagnosing headache type description:Headache attacks that are believed to be a subtypeof trigeminal autonomic cephalalgia but which donot quite meet the diagnostic criteria for any of thesubtypes described above.Diagnosing headache type diagnostic criteria:A. Attacks fulfilling all but one of the specific criteriafor one of the subtypes of trigeminal autonomiccephalalgiaB. Not attributed to another disorderDiagnosing headache type comment:Patients coded as 3.4 Probable trigeminal autonomiccephalalgia or one of its subforms either have had aninsufficient number of typical attacks or fail to fulfilone of the other criteria.3.4.1 Probable cluster headacheDiagnosing headache type diagnostic criteria:A. Attacks fulfilling all but one of criteria AD for3.1 Cluster headacheB. Not attributed to another disorder3.4.2 Probable paroxysmal hemicraniaDiagnosing headache type diagnostic criteria:A. Attacks fulfilling all but one of criteria AE for 3.2Paroxysmal hemicraniaB. Not attributed to another disorder3.4.3 Probable short-lasting unilateral neuralgiformheadache attacks with conjunctival injection andtearingDiagnosing headache type diagnostic criteria:A. Attacks fulfilling all but one of criteria AD for3.3 Short-lasting unilateral neuralgiform headacheattacks with conjunctival injection and tearing(SUNCT)B. Not attributed to another disorderReferencesGeneralGoadsby PJ. Pathophysiology of cluster headache: a trigeminalautonomic cephalgia. Lancet Neurology 2002; 1:3743.Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias,SUNCT syndrome and other short-lasting headaches withautonomic features, including new cases. Brain 1997;120:193209.May A, Goadsby PJ. The trigeminovascular system inhumans: pathophysiological implications for primaryheadache syndromes of the neural influences on the cerebralcirculation. Journal of Cerebral Blood Flow and Metabolism1999; 19:11527.3.1 Cluster headacheAlberca R, Ochoa JJ. Cluster tic syndrome. Neurology 1994;44:9969.Bahra A, May A, Goadsby PJ. Diagnostic patterns in clusterheadache. In: Olesen J, Goadsby PJ, editors. ClusterHeadache and Related Conditions. Oxford: Oxford UniversityPress 1999: pp. 6165.Bahra A, May A, Goadsby PJ. Cluster headache: a prospectiveclinical study in 230 patients with diagnostic implications.Neurology 2002; 58:35461.Bing R. Uber traumatische Erythromelalgie und Erthroprosopalgie.Nervenarzt 1930; 3:50612.de Fine Olivarius B. Hemicrania neuralgiformis chronica(Chronic migrainous neuralgia). Quoted by Sjaastad O, ed.Proceedings of the Scandinavian Migraine Society AnnualMeeting 1971: p. 8.Ekbom K. Ergotamine tartrate orally in Hortons histaminiccephalalgia (also called Harriss ciliary neuralgia). ActaPsychiatrica Scandinavia 1947; 46:106.Ekbom K. Nitroglycerin as a provocative agent in clusterheadache. Archives of Neurology 1968; 19:48793.Eulenberg A. Lehrbuch der Nervenkrankheiten. 2nd edn.Berlin: Hirschwald 1878.Harris W. Ciliary (migrainous) neuralgia and its treatment.British Medical Journal 1936; 1:45760.Horton BT. Histaminic cephalgia. Lancet 1952; ii:928.Kudrow L. Cluster headache: Mechanisms and Management.Oxford: Oxford University Press 1980.Manzoni GC. Gender ratio of cluster headache over the years:a possible role of changes in lifestyle. Cephalalgia 1998;18:13842.Manzoni GC, Micieli G, Granella F, Tassorelli C, Zanferrari C,Diagnosing headache type (IHS 2003)48 ICHD-IICavallini A. Cluster headache-course over ten years in 189patients. Cephalalgia 1991; 11:16974.Manzoni GC, G.Terzano M, Bono G, Micieli G, Martucci N,Nappi G. Cluster headache clinical findings in 180patients. Cephalalgia 1983; 3:2130.May A, Bahra A, Buchel C, Frackowiak RSJ, Goadsby PJ.Hypothalamic activation in cluster headache attacks.Lancet 1998; 351:2758.Mulleners WM, Verhagen WIM. Cluster-tic syndrome. Neurology1996; 47:302.Pascual J, Berciano J. Relief of cluster-tic syndrome by thecombination of lithium and carbamazepine. Cephalalgia1993; 13:2056.Romberg MH. Lehrbuch der Nervenkrankheiten des Menschen.Berlin: Dunker 1840.Russell MB, Andersson PG, Thomsen LL, Iselius L. Clusterheadache is an autosomal dominantly inherited disorder insome families: a complex segregation analysis. Journal ofMedical Genetics 1995; 32:9546.Sjostrand C, Waldenlind E, Ekbom K. A follow up study of 60patients after an assumed first period of cluster headache.Cephalalgia 2000; 20:6537.Sluder G. The syndrome of sphenopalatine ganglion neurosis.American Journal of Medicine 1910; 140:86878.Solomon S, Apfelbaum RI, Guglielmo KM. The cluster-tic syndromeand its surgical therapy. Cephalalgia 1985; 5:839.Torelli P, Cologno D, Cademartiri C, Manzoni GC. Applicationof the International Headache Society classificationcriteria in 652 cluster headache patients. Cephalalgia 2001;21:14550.Vail HH. Vidian neuralgia. Ann Otol Rhinol Laryngol 1932;41:83756.Watson P, Evans R. Cluster-tic syndrome. Headache 1985;25:1236.3.2 Paroxysmal hemicraniaAntonaci F, Pareja JA, Caminero AB, Sjaastad O. Chronicparoxysmal hemicrania and hemicrania continua. Parenteralindomethacin: the Indotest. Headache 1998;38:1228.Antonaci F, Sjaastad O. Chronic paroxysmal hemicrania(CPH): a review of the clinical manifestations. Headache1989; 29:64856.Broeske D, Lenn NJ, Cantos E. Chronic paroxysmal hemicraniain a young child: possible relation to ipsilateral occipitalinfarction. Journal of Child Neurology 1993; 8:2356.Caminero AB, Pareja JA, Dobato JL. Chronic paroxysmal hemicrania-tic syndrome. Cephalalgia 1998; 18:15961.Hannerz J. The second case of chronic paroxysmalhemicrania-tic syndrome [Editorial Comment]. Cephalalgia1998; 18:124.Kudrow DB, Kudrow L. Successful aspirin prophylaxis in achild with chronic paroxysmal hemicrania. Headache 1989;29:2801.Sjaastad O, Dale I. Evidence for a new (?) treatable headacheentity. Headache 1974;14:105108.Zukerman E, Peres MFP, Kaup AO, Monzillo PH, Costa AR.Chronic paroxysmal hemicrania-tic syndrome. Neurology2000; 54:15246.3.3 SUNCTBenoliel R, Sharav Y. Trigeminal neuralgia with lacrimation orSUNCT syndrome? Cephalalgia 1998; 18:8590.Bouhassira D, Attal N, Esteve M, Chauvin M. SUNCT syndrome.A case of transformation from trigeminal neuralgia.Cephalalgia 1994; 14:16870.Bussone G, Leone M, Volta GD, Strada L, Gasparotti R. Shortlastingunilateral neuralgiform headache attacks withtearing and conjunctival injection: the first symptomaticcase. Cephalalgia 1991; 11:1237.De Benedittis G. SUNCT syndrome associated with cavernousangioma of the brain stem. Cephalalgia 1996; 16:5036.Ferrari MD, Haan J, van Seters AP. Bromocriptine-inducedtrigeminal neuralgia attacks in a patient with pituitarytumor. Neurology 1988; 38:14824.Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias,SUNCT syndrome and other short-lasting headaches withautonomic features, including new cases. Brain 1997;120:193209.Goadsby PJ, Matharu MS, Boes CJ. SUNCT syndrome ortrigeminal neuralgia with lacrimation. Cephalalgia 2001;21:823.Levy MJ, Matharu MS, Goadsby PJ. Prolactinomas, dopamineagonist and headache: two case reports. European Journalof Neurology 2003; 10:16974.Massiou H, Launay JM, Levy C, El Amran M, Emperauger B,Bousser M-G. SUNCT syndrome in two patients with prolactinomasand bromocriptine-induced attacks. Neurology2002; 58:16989.Matharu MS, Levy MJ, Merry RT, Goadsby PJ. SUNCT syndromesecondary to prolactinoma. J. Neurol. Neurosurg.Psychiatry 2003; in press.Morales F, Mostacero E, Marta J, Sanchez S. Vascular malformationof the cerebellopontine angle associated withSUNCT syndrome. Cephalalgia 1994; 14:3012.Moris G, Ribacoba R, Solar DN, Vidal JA. SUNCT syndromeand seborrheic dermatitis associated with craneosynostosis.Cephalalgia 2001; 21:1579.Pareja JA, Sjaastad O. SUNCT syndrome. A clinical review.Headache 1997; 37:195202.Penart A, Firth M, Bowen JRC. Short-lasting unilateral neuralgiformheadache with conjunctival injection and tearing(SUNCT) following presumed dorsolateral brainsteminfarction. Cephalalgia 2001; 21:2369.Sjaastad O, Saunte C, Salvesen R, Fredriksen TA, Seim A, RoeOD et al. Shortlasting unilateral neuralgiform headacheattacks with conjunctival injection, tearing, sweating, andrhinorrhea. Cephalalgia 1989; 9:14756.ter Berg HWM, Goadsby PJ. Significance of atypical presentationof symptomatic SUNCT: a case report. J Neurol NeurosurgPsychiat 2001; 70:24446.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 494. Other primary headaches4.1 Primary stabbing headache4.2 Primary cough headache4.3 Primary exertional headache4.4 Primary headache associated with sexualactivity4.4.1 Preorgasmic headache4.4.2 Orgasmic headache4.5 Hypnic headache4.6 Primary thunderclap headache4.7 Hemicrania continua4.8 New daily-persistent headache (NDPH)General commentPrimary or secondary headache or both?When a new headache occurs for the first time inclose temporal relation to another disorder that is aknown cause of headache, this headache is codedaccording to the causative disorder as a secondaryheadache. This is also true if the headache has thecharacteristics of migraine or other primaryheadache. When a pre-existing primary headache ismade worse in close temporal relation to anotherdisorder that is a known cause of headache, thereare two possibilities, and judgment is required. Thepatient can either be given only the diagnosis ofthe pre-existing primary headache or be givenboth this diagnosis and a secondary headache diagnosisaccording to the other disorder. Factorsthat support adding the latter diagnosis are: a veryclose temporal relation to the disorder, a markedworsening of the pre-existing headache, very goodevidence that the disorder can cause or aggravate theprimary headache and, finally, improvement or resolutionof the primary headache after relief from thedisorder.IntroductionThis chapter includes headaches that are clinicallyheterogeneous. The pathogenesis of these types ofheadache is still poorly understood, and their treatmentis suggested on the basis of anecdotal reportsor uncontrolled trials.Several headache disorders included in thischapter can be symptomatic and need careful evaluationby imaging and/or other appropriate tests.The onset of some of these headaches, 4.6 Primarythunderclap headache especially, can be acute andaffected patients are usually assessed in EmergencyDepartments. Appropriate and full investigation(neuroimaging, in particular) is mandatory in thesecases.The chapter also includes some clinical entities,such as 4.1 Primary stabbing headache and 4.5 Hypnicheadache (this latter recently described), that areprimary in most cases.4.1 Primary stabbing headacheDiagnosing headache type previously used terms:Ice-pick pains, jabs and jolts, ophthalmodyniaperiodicaDiagnosing headache type description:Transient and localised stabs of pain in the head thatoccur spontaneously in the absence of organicdisease of underlying structures or of the cranialnerves.Diagnosing headache type diagnostic criteria:A. Head pain occurring as a single stab or a seriesof stabs and fulfilling criteria BDB. Exclusively or predominantly felt in the distributionof the first division of the trigeminal nerve(orbit, temple and parietal area)C. Stabs last for up to a few seconds and recur withirregular frequency ranging from one to manyper dayD. No accompanying symptomsE. Not attributed to another disorder1Note:1. History and physical and neurological examinationsdo not suggest any of the disorders listed ingroups 512, or history and/or physical and/orneurological examinations do suggest such disorderbut it is ruled out by appropriate investigations,or such disorder is present but pain doesnot occur for the first time in close temporal relationto the disorder.Diagnosing headache type comments:In a single published descriptive study, 80% of stabslasted 3 seconds or less. In rare cases, stabs occurrepetitively over days, and there has been onedescription of status lasting one week.Stabs may move from one area to another in eitherthe same or the opposite hemicranium. When theyare strictly localised to one area, structural changesat this site and in the distribution of the affectedcranial nerve must be excluded.Stabbing pains are more commonly experiencedby people subject to migraine (about 40%) or clusterheadache (about 30%), in which cases they are felt inthe site habitually affected by these headaches.Diagnosing headache type (IHS 2003)50 ICHD-IIA positive response to indomethacin has beenreported in some uncontrolled studies, whilst othershave observed partial or no responses.4.2 Primary cough headacheDiagnosing headache type previously used terms:Benign cough headache, Valsalva-manoeuvreheadacheDiagnosing headache type description:Headache precipitated by coughing or straining inthe absence of any intracranial disorder.Diagnosing headache type diagnostic criteria:A. Headache fulfilling criteria B and CB. Sudden onset, lasting from one second to 30minutesC. Brought on by and occurring only in associationwith coughing, straining and/or ValsalvamanoeuvreD. Not attributed to another disorder1Note:1. Cough headache is symptomatic in about 40% ofcases and the large majority of these presentArnold-Chiari malformation type I. Otherreported causes of symptomatic cough headacheinclude carotid or vertebrobasilar diseases andcerebral aneurysms. Diagnostic neuroimagingplays an important role in differentiating secondarycough headache from 4.2 Primary coughheadache.Diagnosing headache type comment:Primary cough headache is usually bilateral and predominantlyaffects patients older than 40 years ofage. Whilst indomethacin is usually effective in thetreatment of primary cough headache, a positiveresponse to this medication has also been reportedin some symptomatic cases.4.3 Primary exertional headacheDiagnosing headache type previously used terms:Benign exertional headacheCoded elsewhere:Exercise-induced migraine is coded under 1.Migraine according to its subtype.Diagnosing headache type description:Headache precipitated by any form of exercise.Subforms such as weight-lifters headache arerecognised.Diagnosing headache type diagnostic criteria:A. Pulsating headache fulfilling criteria B and CB. Lasting from 5 minutes to 48 hoursC. Brought on by and occurring only during or afterphysical exertionD. Not attributed to another disorder1Note:1. On first occurrence of this headache type it ismandatory to exclude subarachnoid haemorrhageand arterial dissection.Diagnosing headache type comments:Primary exertional headache occurs particularly inhot weather or at high altitude. There are reports ofprevention in some patients by the ingestion of ergotaminetartrate. Indomethacin has been found effectivein the majority of the cases.Headache described in weight-lifters has beenconsidered a subform of 4.3 Primary exertionalheadache; because of its sudden onset and presumedmechanism it may have more similarities to 4.2Primary cough headache.4.4 Primary headache associated with sexual activityDiagnosing headache type previously used terms:Benign sex headache, coital cephalalgia, benign vascularsexual headache, sexual headacheDiagnosing headache type description:Headache precipitated by sexual activity, usuallystarting as a dull bilateral ache as sexual excitementincreases and suddenly becoming intense at orgasm,in the absence of any intracranial disorder.4.4.1 Preorgasmic headacheDiagnosing headache type diagnostic criteria:A. Dull ache in the head and neck associated withawareness of neck and/or jaw muscle contractionand fulfilling criterion BB. Occurs during sexual activity and increases withsexual excitementC. Not attributed to another disorder4.4.2 Orgasmic headacheCoded elsewhere:Postural headache resembling that of low CSF pressurehas been reported to develop after coitus. Suchheadache should be coded as 7.2.3 Headache attributedto spontaneous (or idiopathic) low CSF pressurebecause it is due to CSF leakage.Diagnosing headache type (IHS 2003)Diagnosing headache type ICHD-II 51Diagnosing headache type diagnostic criteria:A. Sudden severe (explosive) headache fulfillingcriterion BB. Occurs at orgasmC. Not attributed to another disorder1Note:1. On first onset of orgasmic headache it is mandatoryto exclude conditions such as subarachnoidhaemorrhage and arterial dissection.Diagnosing headache type comments:An association between 4.4 Primary headache associatedwith sexual activity, 4.3 Primary exertional headacheand migraine is reported in approximately 50% ofcases.Two subtypes (dull type and explosive type headache)were included in the first edition of The InternationalClassification of Headache Disorders. No sp |
