A lady with frontal syndrome and a midbrain lesion
"Frontal syndrome and midbrain lesion case report (CPC)"
In some cases a slowly progressive dementia co-exists with frontal lobe syndrome. A lady with frontal syndrome and a midbrain lesion Slideshow transcript
Slide 1: 56 year old, lady, widowed + 3, Syrian descent presented to ER withspeech difficulty, dizziness and general weakness.3 weeks prior to admission, around the time of family bereavement(mother dying of lymphoma aged 87) began to complain of occipitalheadache.At the same time multiple complaints - “blurred vision” unable to define accurately - general weakness, unclear history of mild febrile illness - articulation difficulty - peri-oral paraesthesia - left hand paraesthesia - bilateral arm weakness - left leg weakness AskTheNeurologist.Com Slide 2: Examined in neurology clinic 4 days previously - examination at that time normal - sent for CT ambulatory 2 days prior to admission presented to ER due to worsening of above symptoms - examination normal - treatment commenced with aspirin and patient advised to continue investigation on ambulatory basis CT performed on day prior to admission normal On day of admission worsening of above symptoms as well as a brief episode of diplopia and difficulty swallowing. AskTheNeurologist.Com Slide 3: Past History Mild hypothyroidism S/P R lobectomy for hyperthyroidism Hypercholesterolemia S/P head radiation for tinea capitis On admission receiving aspirin 100mg only A lady with frontal syndrome and a midbrain lesion AskTheNeurologist.Com Slide 4: Examination on admission General examination unremarkableFully conscious and orientatedNo language deficitSpeech mildly dysarthricCranial nerves in tactPower preserved, reflexes symmetrical, no pyramidal signs,Sensory examination in tactNo cerebellar signsGait in tact AskTheNeurologist.Com Slide 5: Neurological examination on dayfollowing admission Fully conscious and orientated Speech: - mild cerebellar dysarthria with nasal quality Upgaze nystagmus. Cranial nerves otherwise in tact Tone, power, reflexes symmetrical and in tact No pyramidal signs Sensory examination in tact Mild cerebellar signs bilaterally Gait mildly ataxic AskTheNeurologist.Com Slide 6: Neurological examination 2 daysfollowing admissionFully conscious and orientated PEARLASkew deviation with no clear cranial nerve lesionRest of examination as abovePsychiatric examination revealed “ normal grief reaction”Treatment with thiamine commenced A lady with frontal syndrome and a midbrain lesion AskTheNeurologist.Com Slide 7: Investigations performed which werenormalBlood count, biochemistryESR ( 17 )LP: - Pressure 17, no cells, TP = 475, OCB –veTumour markersAchR Ab’sB1EBV, CMV – no sign of acute infectionWhole body CT AskTheNeurologist.Com Slide 8: Other Investigations performed Thyroid USS multinodular goitre left lobe, R lobe absentThyroid FNAC consistent with benign multinodular goitreThyroid functionVDRLANAAnti-cardiolipin frontal syndrome AskTheNeurologist.Com Slide 9: MRI at this stage Mass in middle aspect of lower midbrain / junction with pons - 1cm, round, enhancing - Surrounding hyperintense T2 and FLAIR signal - No major distortion of surrounding structuresAdditional small foci - non-enhancing, hyperintense T2 and FLAIR - R pons and L frontal white matter AskTheNeurologist.Com Slide 10: Neurological examination 5 daysfollowing admission General deterioration, complains of worsening headacheAgitationDecreased co-operationBilateral gaze paresis “ eyes fixed” AskTheNeurologist.Com Slide 11: Neurological examination 9 days following admission Deterioration in level of consciousness - Unresponsive to painful stimuli - No eye opening - No vocalisation - Corneal reflexes and Doll’s eyes preserved - not moving arms - spontaneous symmetrical leg movementsHEAD CT – revealed hypodense lesion in midbrainWhole body PET: no evidence of lesions outside of brainTreatment with dexamethasone initiated AskTheNeurologist.Com Slide 12: Neurological examination 12 daysfollowing admissionImprovement in conscious levelOrientated in time and placeMoves all 4 limbs symmetricalNo pyramidal signsFrontal syndrome Over following 2 weeks ( until now ) progressiveimprovement in conscious state and orientationIn parallel with above improvement, development of frontal syndrome AskTheNeurologist.Com Slide 13: Neurological examination 1 monthfollowing admission ( up to date ) Fully conscious and orientatedMinimal co-operation with examination - disinhibited speech - Echolalia - perseveration - hyper-orality - utilisation behaviour - frontal syndrome frontal syndrome AskTheNeurologist.Com Slide 14: Frontal signs continued….. - Pout - Grasp bilaterally - PMR absent bilaterally - Paratonia on right side AskTheNeurologist.Com Slide 15: Neurological examination 1 monthfollowing admission continuedCranial nerves - Skew deviation ( R eye above left ) - No voluntary or pursuit eye movements - Doll’s eyes and caloric responses preservedMoves 4 limbs symmetricallyReflexes slightly brisker on RChadok on RSensation grossly in tactRest of examination limited due to lack of co-operation AskTheNeurologist.Com Slide 16: MRI 6 days ago Mass in middle aspect of midbrain - smaller less enhancing with less oedema In addition to previously noted small subcortical foci: - Bilateral frontal sub-cortical - Non-enhancing AskTheNeurologist.Com Slide 17: Vague symptoms until admission Development of brainstem signs Stable but with severe Recovery of conscious state frontal syndrome Coma: steroids initiated 0 21 23 26 30 32 50 DAYS SINCE SYMPTOM ONSET AskTheNeurologist.Com Slide 18: Summary 56 year old ladyProgressive brainstem syndrome with subsequentdevelopment of a severe frontal syndrome followingimprovement of conscious state under steroidtreatment AskTheNeurologist.Com Slide 19: Upper limb paralysis with movements of lower limbs preserved MIDBRAIN AskTheNeurologist.Com Slide 20: Can MRI findings explain clinicalpicture?Brainstem syndrome including coma readily explained bylesion in lower midbrainCan frontal syndrome be explained on basis of lesions? AskTheNeurologist.Com Slide 21: Clinical syndrome explained by MRI findings YES NO Vasculitic infarcts Infective - Syphilis - CJDMultiple emboli - Whipples - HIVDemyelination Metabolic - Thiamine deficiencyNeoplasia Paraneoplastic - Limbic encephalitis AskTheNeurologist.Com Slide 22: Decreased consciousness…? Amplification of frontal deficit AskTheNeurologist.Com Slide 23: “ Disorders of higher mental function due to single infarctions in thethalamus and in the area of the thalamofrontal tracts” Kalashnikova et al Neuroscience and Behavioural Physiology 1999 9 patients with small infarcts in the thalamus or thalamo-frontal tracts described in which the result was an acute cognitive syndrome. The complex of changes most closely approximated a “frontal syndrome” ; either apathetic, akinetic, or disinhibited type. Lesions were all in positions corresponding to structures traversed by pathways connecting the reticular formation to the frontal lobes Suggest that “frontal syndromes” may be caused by “ functional inactivation of frontal cortex” AskTheNeurologist.Com Slide 24: AskTheNeurologist.Com Slide 25: AskTheNeurologist.Com Slide 26: Clinical syndrome explained by MRI findings YES NO Vasculitic infarcts Infective - Syphilis - CJDMultiple emboli - Whipples - HIVDemyelination Metabolic - Thiamine deficiencyNeoplasia Paraneoplastic - Limbic encephalitis AskTheNeurologist.Com Slide 27: Granulomatous arteritis of the Nervous system( most likely type of vasculitis due to lack of systemic features) For AgainstMultiple subcortical and CSF is often normal butbrainstem lesions during active phase with infarcts inflammatory changes usually foundSubacute courseHeadachesCognitive component oftenprominentResponse to steroids AskTheNeurologist.Com Slide 28: Vasculitis of the nervous systemA.Siva, Journal of Neurology, 2001 AskTheNeurologist.Com Slide 29: AskTheNeurologist.Com Slide 30: AskTheNeurologist.Com Slide 31: AskTheNeurologist.Com Slide 32: Primary CNS Lymphoma For AgainstMay have multifocal Subcortical lesions notappearance characteristicAppearance of midbrain Vasculitis associated withlesion suggestive lymphoma usually occurs with systemic lymphomaIntravascular lymphomamay simulate vasculitisResponse to steroids AskTheNeurologist.Com Slide 33: Localized isolated angiitis of the central nervous system associated withprimary intracerebral lymphoma.Borenstein et al Cancer 1988 When GANS is associated with malignancy, it is usually due to lymphoma outside of the CNS Present case of 52 year-old man presenting with a focal neurological syndrome attributable to the midbrain and headaches. GANS was confirmed via angiography and brain biopsy Good response to treatment initially achieved with cyclophosphamide and steroids Patient subsequently admitted with features of a left occipital lobe infarct Patient died due to complications. Autopsy revealed primary CNS lymphoma in midbrain adjacent to severe area of vasculitis Believe primary disease was lymphoma; but cannot exclude possibility that lymphoma was secondary to cytotoxic treatment AskTheNeurologist.Com Slide 34: Acute Demyelination For AgainstWhite matter lesions on MRI CSF usually abnormalPossible history of mild Clinical syndrome morefebrile illness during severe than MRIprodrome (ADEM) appearance MRI not typical No previous neurological syndrome suggestive of MS No evidence of immunocompromise AskTheNeurologist.Com (PML) Slide 35: Clinical syndrome expalined by MRI findings YES NO Vasculitic infarcts Infective - Syphilis - CJDMultiple emboli - Whipples - HIVDemyelination Metabolic - Thiamine deficiencyNeoplasia Paraneoplastic - Limbic encephalitis AskTheNeurologist.Com Slide 36: Whipple’s disease For AgainstGlobal supranuclear Very rarely occurs withoutopthalmoplegia GIT involvementMost common neurological Frontal syndrome nevermanifestation is cognitive / documentedbehavioural / psychiatricaloneFocal enhancing lesionsmay be present or absentAlthough oculomasticatory and oculo-facial-skeletalmyorhythmia is said to be pathognomonic, it is onlypresent in 20% of cases AskTheNeurologist.Com Slide 37: HIV For AgainstMay cause subacute No risk factorscognitive syndrome withvariable presentations(including predominantlyfrontal syndrome)May cause combination of No other clinical featuresfocal and non-focalfeaturesCSF may be normal.May be combined with otherrelated conditions such as lymphoma, endocarditis ,syphilis or opportunistic infections leading to focalclinical features.AskTheNeurologist.Com Slide 38: Syphilis For AgainstMay cause combination of Sterile CSF during activefocal features ( via disease effectivelyvasculitis) and a more excludeswidespread cognitive diagnosissyndrome ( including frontal)May cause global No other clinical featuressupranuclearopthalmoplegia AskTheNeurologist.Com Slide 39: CJD For AgainstSubacute course Response to steroidsCerebellar signs initially Predominantly frontalfound syndrome rather than dementiaClinical syndrome more Normal EEG initiallysevere than imagingProminent cognitive changes MRI lesions ( without hyperintensity of lenticular nuclei)Normal CSF AskTheNeurologist.Com Slide 40: Limbic encephalitis For AgainstSupranuclear gaze palsies Frontal rather than “limbic syndrome”May cause focal ( including No neoplasm detectedbrainstem) lesions in even with whole-bodyassociation with more PETwidespread cognitivechangesOften associated with Response to steroidscerebellar signs AskTheNeurologist.Com Slide 41: Resident’s summary of DD AskTheNeurologist.Com Slide 42: Vasculitis - GANS most likely. Or related to systemic inflammatory disease Lymphoma Paraneoplastic encephalitis • AngiographyHIV • Brain biopsyCJD • Send full immune screenDemyelination ( ANA, ACL, RF, Anti-Ro, La )Syphilis • Anti Hu?Whipples • VDRL pending • PCR T. Whippelii ( brain, GIT )? AskTheNeurologist.Com Slide 43: Final diagnosis • Angiography show typical beading pattern associated with vasculitis (GANS)• Dramatic improvement following cyclophosphamide treatment• Attained independence after 3 months of treatment AskTheNeurologist.Com
You can ask our panel of neurologists about the frontal syndrome at our neurology advice forum. Some people have noted that the impulsivity that sometimes occurs in the frontal lobe syndrome is similar to some of the features of ADHD , suggesting that the frontal lobes function differently in people with this trait.
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