A prion disease--possible Gerstmann-Straussler-Scheinker disease: a case report.
A 50-year-old patient with a 6-month history of progressive cognitive and motor disability is presented. There were no myoclonic jerks on examination and no periodic sharp waves by electroencephalography. Imaging showed high signal on T2-weighted scans in the basal ganglia and posterior limbs of the internal capsules, with no restricted diffusion and parenchymal volume loss. A brain biopsy was performed. Western blot analysis revealed a protease-resistant prion protein fragment (PrP7-8), the molecular hallmark of Gerstmann-Straussler-Scheinker disease.
Aralasmak
Ayse
A
Russell H. Morgan Department of Radiology and Radiological Science, Division of Neuroradiology, Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA. aysearalasmak@hotmail.com
Crain
Barbara J
BJ
Zou
Wen-Quan
WQ
Yousem
David M
DM
D015153:Blotting, Western; D003937:Diagnosis, Differential; D004569:Electroencephalography; D016098:Gerstmann-Straussler-Scheinker Disease / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged