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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
PseudotumorPseudotumor
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
Creutzfeld-Jakob DiseaseCreutzfeld-Jakob Disease
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
Creutzfeld-Jakob DiseaseCreutzfeld-Jakob Disease
““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
Creutzfeld-Jakob DiseaseCreutzfeld-Jakob Disease
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
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
PseudotumorPseudotumor
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
Creutzfeld-Jakob DiseaseCreutzfeld-Jakob Disease
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
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