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急性暴发性亚急性硬化性全脑炎:临床及脑电图特征

Acute fulminant SSPE: clinical and EEG features.

作者信息

Gökçil Z, Odabaşi Z, Aksu A, Vural O, Yardim M

机构信息

Department of Neurology, Gülhane Medical School, Ankara, Turkey.

出版信息

Clin Electroencephalogr. 1998 Jan;29(1):43-8. doi: 10.1177/155005949802900112.

DOI:10.1177/155005949802900112
PMID:9472425
Abstract

We report an autopsy-proven case of SSPE in which the course was rapidly progressive, leading to death in 2 months. The patient was admitted for investigation of visual symptoms. His examination revealed complete right homonymous hemianopia and myoclonic jerks in the right half of the body. EEG showed high voltage periodic slow-wave discharges that were often associated with jerks. Measles antibody was positive in serum and CSF. MRI showed hyperintensity in the white matter of the occipital poles. The patient became completely blind and had jerks in all four limbs. His condition continued to deteriorate and within 3 weeks he reached a state of deep coma. The myoclonic jerks became less marked and gradually completely ceased. The EEG showed diffuse slow activity without periodic complexes. The patient died approximately 2 months from onset of disease. Brain autopsy revealed severe neuronal loss with abundant inclusion bodies in the remaining neuronal and oligodendroglial nuclei, which was compatible with SSPE.

摘要

我们报告一例经尸检证实的亚急性硬化性全脑炎(SSPE)病例,其病程呈快速进展,在2个月内导致死亡。该患者因视觉症状入院检查。检查发现其有完全性右侧同向性偏盲及右侧半身肌阵挛性抽搐。脑电图显示高电压周期性慢波放电,常伴有抽搐。血清和脑脊液中的麻疹抗体呈阳性。磁共振成像显示枕叶白质高信号。患者完全失明,四肢均出现抽搐。其病情持续恶化,3周内进入深度昏迷状态。肌阵挛性抽搐变得不那么明显并逐渐完全停止。脑电图显示弥漫性慢活动,无周期性复合波。患者自疾病发作起约2个月后死亡。脑部尸检显示严重的神经元丢失,在剩余的神经元和少突胶质细胞核中有大量包涵体,这与亚急性硬化性全脑炎相符。

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引用本文的文献

1
A rare presentation of subacute sclerosing panencephalitis with acute fulminant course and atypical radiological features.一例具有急性暴发性病程和非典型影像学特征的亚急性硬化性全脑炎罕见病例。
Ann Indian Acad Neurol. 2013 Oct;16(4):732-3. doi: 10.4103/0972-2327.120437.
2
Fulminant adult-onset subacute sclerosing panencephalitis: a case report.暴发性成人起病亚急性硬化性全脑炎:一例报告
BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.09.2008.0922. Epub 2009 Mar 2.
3
Subacute sclerosing panencephalitis.亚急性硬化性全脑炎
Postgrad Med J. 2002 Feb;78(916):63-70. doi: 10.1136/pmj.78.916.63.