伴有阿尔茨海默病病理特征的克雅氏病，反复部分性癫痫发作后出现癫痫持续状态：一例报告及文献综述
Creutzfeldt-Jakob disease with Alzheimer pathology, presenting with status epilepticus following repeated partial seizures: a case report and literature review.
作者信息
Miyake Keita, Hara Takashi, Oshima Etsuko, Kawada Kiyohiro, Ishizu Hideki, Yamauchi Yuko, Satoh Katsuya, Kitamoto Tetsuyuki, Takenoshita Shintaro, Terada Seishi, Yamada Norihito
机构信息
Department of Psychiatry, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama, 700-8558, Japan.
Department of Psychiatry, Hayashi Hospital, Okayama, Japan.
出版信息
BMC Neurol. 2018 Apr 25;18(1):54. doi: 10.1186/s12883-018-1055-y.
BACKGROUND
Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disease. Common first symptoms are dementia, cerebellar ataxia, visual disturbance, and psychiatric symptoms. Seizure as the first symptom of CJD is a very rare finding.
CASE PRESENTATION
We experienced an elderly woman who presented initially with status epilepticus following repeated partial seizures in the course of Alzheimer disease (AD) dementia. Anti-convulsive therapy had no effect. Autopsy revealed definite CJD with AD pathology.
COCLUSIONS
This is the first reported CJD case presenting with status epilepticus in the course of AD dementia.
背景
克雅氏病（CJD）是一种致命的神经退行性疾病。常见的首发症状是痴呆、小脑共济失调、视觉障碍和精神症状。癫痫发作作为CJD的首发症状是非常罕见的表现。
病例报告
我们接诊了一位老年女性，她最初在阿尔茨海默病（AD）痴呆病程中反复出现部分性癫痫发作后发展为癫痫持续状态。抗惊厥治疗无效。尸检显示确诊为CJD合并AD病理改变。
结论
这是首例报道的在AD痴呆病程中以癫痫持续状态为表现的CJD病例。
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