Asymmetric involvement in sporadic creutzfeldt-jakob disease: clinical, brain imaging, and electroencephalographic studies.
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.
Wu
Hung-Ming
HM
Department of Neurology, Chang Gung Memorial Hospital at Linkou and College of Medicine, Chang Gung University, Taipei, Taiwan, ROC.
Lu
Chin-Song
CS
Huang
Chin-Chang
CC
Lyu
Rong-Kuo
RK
Chen
Rou-Shayn
RS
Wu
Yih-Ru
YR
Chen
Yi-Chun
YC
Lai
Szu-Chia
SC
Hung
June
J
Tsai
Yu-Tai
YT
Toh
Cheng-Hong
CH
Kuo
Hung-Chou
HC
D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000473:pathology* / Q000503:physiopathology; D001931:Brain Mapping; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology / Q000503:physiopathology*; D038524:Diffusion Magnetic Resonance Imaging; D004569:Electroencephalography / Q000379:methods; D005260:Female; D007839:Functional Laterality / Q000502:physiology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013223:Statistics as Topic