Neurology Potpourri

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Information about Neurology Potpourri
Health & Medicine

Published on February 6, 2009

Author: MedicineAndHealthNeurolog

Source: slideshare.net

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

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

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

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

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

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

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

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.

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

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

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

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

“ 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

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

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

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

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

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

Confusion and inattention

Amnesia

Truncal ataxia

Ophthalmoplegia – usually CN VI

Korsakoff’s Amnesia Attention appears normal, but pt’s confabulate

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

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

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

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