A prion disease - Possible gerstmann-straussler-scheinker disease: A case report

Ayse Aralasmak, Barbara J. Crain, Wen Quan Zou, David M. Yousem

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)135-139
Number of pages5
JournalJournal of computer assisted tomography
Volume30
Issue number1
DOIs
StatePublished - Jan 1 2006

Keywords

  • Gerstmann-Straussler-Scheinker
  • Magnetic resonance imaging findings
  • Prion diseases

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

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