Asymmetric Involvement in Sporadic Creutzfeldt-Jakob Disease: Clinical, Brain Imaging, and Electroencephalographic Studies

作者：Wu Hung Ming; Lu Chin Song; Huang Chin Chang; Lyu Rong Kuo; Chen Rou Shayn; Wu Yih Ru; Chen Yi Chun; Lai Szu Chia; Hung June; Tsai Yu Tai; Toh Cheng Hong; Kuo Hung Chou^*

来源：European Neurology, 2010, 64(2): 74-79.

DOI：10.1159/000315148

• Creutzfeldt-Jakob disease
• Asymmetric involvement
• Brain magnetic resonance imaging
• Electroencephalography
• Periodic sharp wave complex

Objective: To ascertain the characteristics of patients with sporadic Creutzfeldt-Jakob disease (CJD) and to determine the findings of electroencephalography (EEG) and brain magnetic resonance imaging (MRI). Methods: We pooled patients at a hospital from 2000 to 2008, and classified them according to WHO diagnostic criteria as having probable or possible CJD. We retrospectively analyzed their clinical manifestations, brain MRI, and EEG findings to evaluate correlations among them. Results: In this study, 12 probable and 4 possible CJD patients were identified. Ten patients with probable CJD had asymmetric manifestations with hemiparesis, focal myoclonus, dystonia or apraxia; 9 had clinical manifestations mimicking the corticobasal syndrome. In contrast, neurological examinations did not show asymmetric signs in 4 patients with possible CJD. EEG showed a typical periodic sharp wave complex (PSWC) in 12 patients with probable CJD; most of them had bright signal intensity on diffusion-weighted imaging of the cortex and/or basal ganglia. There was a high tendency for asymmetric clinical manifestations that correlated with the presentation of PSWC and cortical lesions observed on the brain MRI scan. Conclusions: Our study indicates that asymmetric extrapyramidal symptoms/signs, in clinical features with characteristic abnormalities on MRI and EEG findings, might contribute to early diagnosis of sporadic CJD.

• 出版日期2010
• 单位长春大学