期刊
EUROPEAN JOURNAL OF NEUROLOGY
卷 18, 期 7, 页码 999-1002出版社
WILEY-BLACKWELL
DOI: 10.1111/j.1468-1331.2010.03185.x
关键词
akinetic mutism state; Creutzfeldt-Jakob disease; MM1-type; Myoclonus; Periodic sharp-wave complexes
资金
- Research Committee on Prion Diseases and Slow Virus Infection, Ministry of Health, Welfare, and Labor, Japan
- Grants-in-Aid for Scientific Research [22249034] Funding Source: KAKEN
Background: It is not known whether the clinical course of Japanese sporadic Creutzfeldt-Jakob disease (sCJD) cases differs from that of Caucasian sCJD cases. Patients and methods: To investigate the clinical course of Japanese sCJD, clinical findings from 29 patients with Japanese MM1-type sCJD were retrospectively evaluated and compared to Caucasian sCJD findings. Results: Survival of Japanese MM1-type sCJD up to the time of akinetic mutism state is similar to that of Caucasian subjects. However, the total disease duration of Japanese patients was approximately three times longer. Conclusions: The present observations indicate that Japanese sCJD cases generally show a longer disease duration because of the longer survival period after reaching the akinetic mutism state.
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