磁共振成像在克雅氏病诊断中的应用：两例报告。

Valente Alan Peres, Pinho Paula da Cunha, Lucato Leandro Tavares

Setor de Neurorradiologia Diagnóstica. Instituto de Radiologia do Hospital das Clínicas da Faculdade de Medicina da USP.

Dement Neuropsychol. 2015 Oct-Dec;9(4):424-427. doi: 10.1590/1980-57642015DN94000424.

Creutzfeldt-Jacob disease (CJD) is a rare condition caused by a pathogenic prion protein that evolves with rapidly progressive dementia and death. The clinical presentation may sometimes be misleading. Magnetic Resonance Imaging (MRI) aids diagnosis with patterns that can guide or confirm clinical hypotheses. Two cases of rapidly progressive dementia with ataxia, myoclonus and restricted diffusion on MRI in cortical/basal ganglia are presented to draw attention to CJD.

克雅氏病（CJD）是一种由致病性朊蛋白引起的罕见疾病，其病情发展迅速，会导致进行性痴呆和死亡。临床表现有时可能具有误导性。磁共振成像（MRI）通过特定模式辅助诊断，这些模式可指导或证实临床诊断假设。本文报告两例患有共济失调、肌阵挛且MRI显示皮质/基底神经节扩散受限的快速进行性痴呆病例，以引起对克雅氏病的关注。