CSF positive for Protease-Resistant PrP7-8 - USMLE Forums
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  #1  
Old 10-23-2011
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Neuro CSF positive for Protease-Resistant PrP7-8

A 50-year-old man presented with 6 months of progressive difficulty in word finding and language output, fine tremors, dysphagia, dysarthria, hoarseness, memory loss, gait difficulties, disorder in movement of his upper extremities, incontinence, decreased appetite, and weight loss. Neurologic examination revealed increase in muscle tone, diffuse fine tremor, hyperreflexia, and unsteady gait. Cerebellar tests were difficult to assess because of the tremor. The result of a cerebrospinal fluid 14-3-3 protein assay was negative, but it was positive for presence of protease-resistant PrP7-8. Electroencephalography showed no specific abnormalities. In particular, there were no seizures or periodic sharp waves. Electromyography and nerve conduction studies were limited due to the presence of tremors; however, the findings demonstrated normal motor unit potentials with no evidence of fibrillation or fasciculation. Histopathology results from the CNS biopsy showed degeneration in the ascending and descending columns and cerebellar dentate nuclei. The MRI imaging findings demonstrated diffuse volume loss in the cerebrum, cerebellum and basal ganglia. What is the most likely diagnosis?

A) Amyotrophic Lateral Sclerosis
B) Sporadic Creutzfeldt-Jakob Disease
C) Fatal Familial Insomnia
D) Gerstmann-Straussler-Scheinker Disease
E) Vascular Dementia
F) Kuru
G) Progressive Multifocal Leukorncephalopathy
H) Subacute Sclerosing Panencephalitis
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Old 10-23-2011
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seems like d) , but not sure
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Old 10-23-2011
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i go for b
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Old 10-23-2011
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how abt G!!
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Old 10-24-2011
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wow.. .. I am taking b, c, d and f out...
h is out too..


hmm a wouldn't cause memory loss....

gahhh hmm g or e? ok which one is it.. inbox?
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Old 10-24-2011
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Correct Answer Answer!

All right guys,

The answer is D.
GSS is characterized clinically by progressive ataxia, parkinsonian symptoms, and dementia and biochemically by the presence of protease-resistant PrP7-8 in the central nervous system. Histopathologically, degeneration has been shown in the ascending and descending columns and cerebellar dentate nuclei. The usual imaging findings for GSS are volume loss in the cerebellum or diffuse volume loss in the cerebrum and cerebellum and, rarely, decreased T2-weighted signal changes in the basal ganglia.

For further explanation and differentiation from other Prion diseases see the following case report:
http://neuroradiology.rad.jhmi.edu/Y...%20Disease.pdf
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