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Washington University Experience | PRION DISEASES | Prion Diseases | 24A1 CJD (Case 24)

Case 24 ---- This 66 year old female presented with a 2.5 month history of progressive neurologic disabilities including ataxia, blurred vision and dizziness. She also complained of blurred vision. Upon examination, although she had a staggering gait, the Romberg test was normal, and there was no past-pointing. Special examinations included cerebral and brachial arteriography, pneumo-encephalography, brain scanning and brain biopsy which was essentially negative. During her hospital stay, she developed bilateral cortical blindness, retrograde behavior, and decerebrate posturing. No ·diagnosis was made, although many were entertained, and no specific treatment was instituted. ---- The brain weighed 1100 grams. However, on coronal section, the ventricles were not enlarged. Microscopically, the occipital region showed neuronal loss and cortical and subcortical astrogliosis, The final diagnosis was subacute spongiform degeneration of the frontal and occipital lobes. (One variant of the original description was reported by Heidenhain).

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