A prion disease--possible Gerstmann-Straussler-Scheinker disease: a case report
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Updated: Aug 14, 2026

Prion Safety Laboratory Swipe Test
Published on: February 14, 2025
Ayse Aralasmak1, Barbara J Crain, Wen-Quan Zou
1Russell H. Morgan Department of Radiology and Radiological Science, Division of Neuroradiology, Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA. aysearalasmak@hotmail.com
Abstract:
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.
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