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Neurology Potpourri
 

Neurology Potpourri

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    Neurology Potpourri Neurology Potpourri Presentation Transcript

    • Idiopathic Intracranial Hypertension (pseudotumor cerebri)
      • obese women of childbearing age
      • Presentation
        • HA: daily, non-throbbing, worse with straining
        • visual field defects
        • “ pulsatile” tinnitus
        • papilledema
      • Etiology
        • Unclear, may be related to decreased CSF resorption
      • Drugs
        • tetracycline, OCP’s, and hypervitaminosis A
    • Pseudotumor
      • Diagnosis of exclusion
        • Need neuroimaging to r/o IC pathology
        • Diagnostic LP with opening pressure
      • Dandy Criteria
        • Signs & symptoms of increased ICP
        • CSF pressure >25 cm water
        • No localizing signs (with the exception of CN VI palsy)
        • Normal CSF composition
        • Normal ventricles on imaging with no intracranial mass
    • Pseudotumor
      • Treatment
        • CA-inhibitors
        • Shunt
      • Permanent visual loss may result if left untreated
    • Delirium
      • Acute, fluctuating alteration in consciousness, attention, and/or cognition
      • May be life-threatening
      • Excellent NEJM Review Article
        • http://content.nejm.org/cgi/reprint/354/11/1157.pdf
      • Excellent algorithm
        • http://content.nejm.org/cgi/data/354/11/1157/DC1/1
    • Reversible/Medical Causes of Delirium
      • M eds, MI
      • I nfection
      • C ortisol
      • U remia
      • B 1,12
      • A BG (low O 2 or high CO 2 )
      • A mmonia
      • R PR
      • T SH
    • Wernicke’s Syndrome
      • Confusion and inattention
      • Amnesia
      • Truncal ataxia
      • Abnormal eye movements
        • Horizontal nystagmus
        • Disconjugate gaze
        • Ophthalmoplegia – usually CN VI
    • Korsakoff’s
      • On a spectrum with Wernicke’s
      • May emerge while treating Wernicke’s
      • Amnesia
      • Attention appears normal, but pt’s confabulate
    • Etiology
      • Thiamine pyrophosphate (TPP), is vital in the metabolism of carbohydrates
        • conversion of pyruvate to acetyl coenzyme A by pyruvate dehydrogenase
        • conversion of α -ketoglutarate to succinate by α -ketoglutarate dehydrogenase in the Krebs cycle
        • catalysis by transketolase in the pentose monophosphate shunt.
      • In the presence of thiamine deficiency, these cellular systems dependent on thiamine begin to fail, leading eventually to cell death.
    • Treatment
      • Thiamine 50mg IV + 50mg IM before any glucose is given by any route
      • Supplement with 50mg IM daily until eating normal diet
      • ~80% effective in early treatment of Wernicke’s
      • ~50% when Korsakoff’s syndrome has developed
    • Creutzfeld-Jakob Disease
      • Infectious protein that cause degenerative CNS disease
      • Incidence: ~1 in 1,000,000
      • Age 17-83, but most 50-75
      • Relentless, rapid progressive dementia
      • Myoclonus (90%) which persists through sleep
      • Exaggerated startle reflex
      • Death within 1 year of symptom onset
    • Creutzfeld-Jakob Disease
      • Sporadic CJD accounts for 85% of prion diseases
      • Inherited form ~10%
      • ~5% “other”
        • kuru
        • scrapie
        • BSE
        • Fatal Familial Insomnia
    • Creutzfeld-Jakob Disease
      • “ Reproduction” occurs by binding of activated protein to the normal cellular isoform of the prion protein (PrP C )
      • α -helix converted to β -pleated sheet, forming the disease-causing isoform of the prion protein (PrP Sc )
      • Transmissable, but not contagious
    • Creutzfeld-Jakob Disease
      • Dementia, myoclonus, and periodic sharp electrical spikes on EEG in an afebrile middle-age or older person are highly suggestive of the disease
      • 14-3-3 is a non-specific protein which may help in diagnosis if found in otherwise normal CSF
      • Sporadic CJD can be confirmed by sequencing of the PRNP gene
    • …In Summary
    • Idiopathic Intracranial Hypertension (pseudotumor cerebri)
      • obese women of childbearing age
      • Presentation
        • HA: daily, non-throbbing, worse with straining
        • visual field defects
        • “ pulsatile” tinnitus
        • papilledema
      • Etiology
        • Unclear, may be related to decreased CSF resorption
    • Pseudotumor
      • Diagnosis of exclusion
        • Need neuroimaging to r/o IC pathology
        • Diagnostic LP with opening pressure
      • Dandy Criteria
        • Signs & symptoms of increased ICP
        • CSF pressure >25 cm water
        • No localizing signs (with the exception of CN VI palsy)
        • Normal CSF composition
        • Normal ventricles on imaging with no intracranial mass
    • Pseudotumor
      • Treatment
        • CA-inhibitors
        • Shunt
      • Permanent visual loss may result if left untreated
    • Reversible/Medical Causes of Delirium
      • M eds, MI
      • I nfection
      • C ortisol
      • U remia
      • B 1,12
      • A BG (low O 2 or high CO 2 )
      • A mmonia
      • R PR
      • T SH
    • Wernicke’s Syndrome
      • Confusion and inattention
      • Amnesia
      • Truncal ataxia
      • Ophthalmoplegia – usually CN VI
    • Korsakoff’s
      • Amnesia
      • Attention appears normal, but pt’s confabulate
    • Treatment
      • Thiamine 50mg IV + 50mg IM before any glucose is given by any route
      • Supplement with 50mg IM daily until eating normal diet
    • Creutzfeld-Jakob Disease
      • Dementia, myoclonus, and periodic sharp electrical spikes on EEG in an afebrile middle-age or older person are highly suggestive of the disease
      • 14-3-3 is a non-specific protein which may help in diagnosis if found in otherwise normal CSF
      • Sporadic CJD can be confirmed by sequencing of the PRNP gene