A prion disease - Possible Gerstmann-Straussler-Scheinker disease - A case report

被引：3

作者：

Aralasmak, A

Crain, BJ

Zou, WQ

Yousem, DM

机构：

[1] Johns Hopkins Med Inst, Russell H Morgan Dept Radiol & Radiol Sci, Div Neuroradiol, Baltimore, MD 21205 USA

[2] Johns Hopkins Univ, Sch Med, Dept Pathol, Baltimore, MD 21205 USA

[3] Case Western Reserve Univ, Inst Pathol, Div Neuropathol, Cleveland, OH 44106 USA

来源：

JOURNAL OF COMPUTER ASSISTED TOMOGRAPHY | 2006年 / 30卷 / 01期

关键词：

prion diseases; Gerstmann-Straussler-Scheinker; magnetic resonance imaging findings;

D O I：

10.1097/01.rct.0000193816.42051.1a

中图分类号：

R8 [特种医学]; R445 [影像诊断学];

学科分类号：

1002 ; 100207 ; 1009 ;

摘要：

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.

引用

页码：135 / 139

页数：5

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