neurology potpourri

Idiopathic Intracranial Hypertension Idiopathic Intracranial Hypertension (pseudotumor cerebri)(pseudotumor cerebri)

obese women of childbearing age obese women of childbearing age

PresentationPresentation HA: daily, non-throbbing, worse with strainingHA: daily, non-throbbing, worse with straining visual field defectsvisual field defects ““pulsatile” tinnituspulsatile” tinnitus papilledemapapilledema

EtiologyEtiology Unclear, may be related to decreased CSF resorptionUnclear, may be related to decreased CSF resorption

DrugsDrugs tetracycline, OCP’s, and hypervitaminosis Atetracycline, OCP’s, and hypervitaminosis A

PseudotumorPseudotumor

Diagnosis of exclusionDiagnosis of exclusion Need neuroimaging to r/o IC pathologyNeed neuroimaging to r/o IC pathology Diagnostic LP Diagnostic LP withwith opening pressure opening pressure

Dandy CriteriaDandy Criteria Signs & symptoms of increased ICP Signs & symptoms of increased ICP CSF pressure >25 cm waterCSF pressure >25 cm water No localizing signs (with the exception of CN VI palsy)No localizing signs (with the exception of CN VI palsy) Normal CSF compositionNormal CSF composition Normal ventricles on imaging with no intracranial massNormal ventricles on imaging with no intracranial mass


TreatmentTreatment CA-inhibitorsCA-inhibitors Shunt Shunt

Permanent visual loss may result if left untreatedPermanent visual loss may result if left untreated

DeliriumDelirium

Acute, fluctuating alteration in consciousness, Acute, fluctuating alteration in consciousness, attention, and/or cognitionattention, and/or cognition

May be life-threateningMay be life-threatening

Excellent NEJM Review ArticleExcellent NEJM Review Article http://content.nejm.org/cgi/reprint/354/11/1157.pdfhttp://content.nejm.org/cgi/reprint/354/11/1157.pdf

Excellent algorithmExcellent algorithm http://content.nejm.org/cgi/data/354/11/1157/DC1/1http://content.nejm.org/cgi/data/354/11/1157/DC1/1

Reversible/Medical Causes of Delirium Reversible/Medical Causes of Delirium

MMeds, MIeds, MI IInfectionnfectionCCortisolortisolUUremiaremia

BB 1,121,12

AABG (low OBG (low O22 or high CO or high CO22))

AAmmoniammoniaRRPRPRTTSHSH

Wernicke’s SyndromeWernicke’s Syndrome

Confusion and inattentionConfusion and inattention

AmnesiaAmnesia

Truncal ataxiaTruncal ataxia

Abnormal eye movementsAbnormal eye movements Horizontal nystagmusHorizontal nystagmus Disconjugate gazeDisconjugate gaze Ophthalmoplegia – usually CN VIOphthalmoplegia – usually CN VI

Korsakoff’sKorsakoff’s

On a spectrum with Wernicke’sOn a spectrum with Wernicke’s

May emerge while treating Wernicke’sMay emerge while treating Wernicke’s

AmnesiaAmnesia

Attention appears normal, but pt’s Attention appears normal, but pt’s confabulateconfabulate

EtiologyEtiology

Thiamine pyrophosphate (TPP), is vital in the Thiamine pyrophosphate (TPP), is vital in the metabolism of carbohydratesmetabolism of carbohydrates

conversion of pyruvate to acetyl coenzyme A by pyruvate conversion of pyruvate to acetyl coenzyme A by pyruvate dehydrogenasedehydrogenase

conversion of conversion of αα-ketoglutarate to succinate by -ketoglutarate to succinate by αα-ketoglutarate -ketoglutarate dehydrogenase in the Krebs cycledehydrogenase in the Krebs cycle

catalysis by transketolase in the pentose monophosphate shunt. catalysis by transketolase in the pentose monophosphate shunt.

In the presence of thiamine deficiency, these cellular In the presence of thiamine deficiency, these cellular systems dependent on thiamine begin to fail, leading systems dependent on thiamine begin to fail, leading eventually to cell death.eventually to cell death.

TreatmentTreatment

Thiamine 50mg IV + 50mg IM before any glucose is Thiamine 50mg IV + 50mg IM before any glucose is given by any routegiven by any route

Supplement with 50mg IM daily until eating normal dietSupplement with 50mg IM daily until eating normal diet

~80% effective in early treatment of Wernicke’s~80% effective in early treatment of Wernicke’s

~50% when Korsakoff’s syndrome has developed~50% when Korsakoff’s syndrome has developed

Creutzfeld-Jakob DiseaseCreutzfeld-Jakob Disease

Infectious proteinInfectious protein that cause degenerative CNS disease that cause degenerative CNS disease

Incidence: ~1 in 1,000,000Incidence: ~1 in 1,000,000

Age 17-83, but most 50-75Age 17-83, but most 50-75

Relentless, rapid progressive Relentless, rapid progressive dementia dementia

Myoclonus Myoclonus (90%)(90%) which persists through sleepwhich persists through sleep

Exaggerated startleExaggerated startle reflex reflex

Death within 1 year of symptom onsetDeath within 1 year of symptom onset


Sporadic CJD accounts for 85% of prion diseasesSporadic CJD accounts for 85% of prion diseases

Inherited form ~10%Inherited form ~10%

~5% “other”~5% “other” kurukuru scrapiescrapie BSEBSE Fatal Familial InsomniaFatal Familial Insomnia


““Reproduction” occurs by binding of activated protein to Reproduction” occurs by binding of activated protein to the normal cellular isoform of the prion protein (PrPthe normal cellular isoform of the prion protein (PrPCC))

αα-helix converted to -helix converted to ββ-pleated sheet, forming the -pleated sheet, forming the disease-causing isoform of the prion protein (PrPdisease-causing isoform of the prion protein (PrPScSc))

Transmissable, but not contagiousTransmissable, but not contagious


Dementia, myoclonus, and periodic sharp electrical Dementia, myoclonus, and periodic sharp electrical spikes on EEG in an afebrile middle-age or older person spikes on EEG in an afebrile middle-age or older person are highly suggestive of the diseaseare highly suggestive of the disease

14-3-314-3-3 is a non-specific protein which may help in is a non-specific protein which may help in diagnosis if found in otherwise normal CSFdiagnosis if found in otherwise normal CSF

Sporadic CJD can be confirmed by sequencing of the Sporadic CJD can be confirmed by sequencing of the PRNPPRNP gene gene

……In SummaryIn Summary

Idiopathic Intracranial Hypertension Idiopathic Intracranial Hypertension (pseudotumor cerebri)(pseudotumor cerebri)

obese women of childbearing age obese women of childbearing age

PresentationPresentation HA: daily, non-throbbing, worse with strainingHA: daily, non-throbbing, worse with straining visual field defectsvisual field defects ““pulsatile” tinnituspulsatile” tinnitus papilledemapapilledema

EtiologyEtiology Unclear, may be related to decreased CSF resorptionUnclear, may be related to decreased CSF resorption


Diagnosis of exclusionDiagnosis of exclusion Need neuroimaging to r/o IC pathologyNeed neuroimaging to r/o IC pathology Diagnostic LP Diagnostic LP withwith opening pressure opening pressure

Dandy CriteriaDandy Criteria Signs & symptoms of increased ICP Signs & symptoms of increased ICP CSF pressure >25 cm waterCSF pressure >25 cm water No localizing signs (with the exception of CN VI palsy)No localizing signs (with the exception of CN VI palsy) Normal CSF compositionNormal CSF composition Normal ventricles on imaging with no intracranial massNormal ventricles on imaging with no intracranial mass


TreatmentTreatment CA-inhibitorsCA-inhibitors Shunt Shunt

Permanent visual loss may result if left untreatedPermanent visual loss may result if left untreated

Reversible/Medical Causes of Delirium Reversible/Medical Causes of Delirium

MMeds, MIeds, MI IInfectionnfectionCCortisolortisolUUremiaremia

BB 1,121,12

AABG (low OBG (low O22 or high CO or high CO22))

AAmmoniammoniaRRPRPRTTSHSH

Wernicke’s SyndromeWernicke’s Syndrome

Confusion and inattentionConfusion and inattention

AmnesiaAmnesia

Truncal ataxiaTruncal ataxia

Ophthalmoplegia – usually CN VIOphthalmoplegia – usually CN VI

Korsakoff’sKorsakoff’s

AmnesiaAmnesia

Attention appears normal, but pt’s Attention appears normal, but pt’s confabulateconfabulate

TreatmentTreatment

Thiamine 50mg IV + 50mg IM before any glucose is Thiamine 50mg IV + 50mg IM before any glucose is given by any routegiven by any route

Supplement with 50mg IM daily until eating normal dietSupplement with 50mg IM daily until eating normal diet


Dementia, myoclonus, and periodic sharp electrical Dementia, myoclonus, and periodic sharp electrical spikes on EEG in an afebrile middle-age or older person spikes on EEG in an afebrile middle-age or older person are highly suggestive of the diseaseare highly suggestive of the disease

14-3-314-3-3 is a non-specific protein which may help in is a non-specific protein which may help in diagnosis if found in otherwise normal CSFdiagnosis if found in otherwise normal CSF

Sporadic CJD can be confirmed by sequencing of the Sporadic CJD can be confirmed by sequencing of the PRNPPRNP gene gene

neurology potpourri

Health & Medicine