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Neurology Potpourri

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Neurology Potpourri

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

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