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类固醇反应性昏睡性脑炎综合征伴恶性紧张症

Steroid-responsive encephalitis lethargica syndrome with malignant catatonia.

作者信息

Ono Yoichi, Manabe Yasuhiro, Hamakawa Yoshiyuki, Omori Nobuhiko, Abe Koji

机构信息

Department of Neurology, National Hospital Organization Okayama Medical Center.

出版信息

Intern Med. 2007;46(6):307-10. doi: 10.2169/internalmedicine.46.6179. Epub 2007 Mar 15.

DOI:10.2169/internalmedicine.46.6179
PMID:17379999
Abstract

We report a 47-year-old man who is considered to have sporadic encephalitis lethargica (EL). He presented with hyperpyrexia, lethargy, akinetic mutism, and posture of decorticate rigidity following coma and respiratory failure. Intravenous methylprednisolone pulse therapy improved his condition rapidly and remarkably. Electroencephalography (EEG) showed severe diffuse slow waves of bilateral frontal dominancy, and paralleled the clinical course. Our patient fulfilled the diagnostic criteria for malignant catatonia, so we diagnosed secondary malignant catatonia due to EL syndrome. The effect of corticosteroid treatment remains controversial in encephalitis; however, some EL syndrome patients exhibit an excellent response to corticosteroid treatment. Therefore, EL syndrome may be secondary to autoimmunity against deep grey matter. It is important to distinguish secondary catatonia due to general medical conditions from psychiatric catatonia and to choose a treatment suitable for the medical condition.

摘要

我们报告了一名47岁的男性,他被认为患有散发性昏睡性脑炎(EL)。他出现高热、嗜睡、运动不能性缄默症,昏迷和呼吸衰竭后出现去皮层强直姿势。静脉注射甲基强的松龙冲击疗法迅速且显著地改善了他的病情。脑电图(EEG)显示双侧额叶为主的严重弥漫性慢波,与临床病程平行。我们的患者符合恶性紧张症的诊断标准,因此我们诊断为EL综合征继发的继发性恶性紧张症。在脑炎中,皮质类固醇治疗的效果仍存在争议;然而,一些EL综合征患者对皮质类固醇治疗表现出良好反应。因此,EL综合征可能继发于针对深部灰质的自身免疫。将一般内科疾病所致的继发性紧张症与精神性紧张症区分开来,并选择适合病情的治疗方法很重要。

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Steroid-responsive encephalitis lethargica syndrome with malignant catatonia.类固醇反应性昏睡性脑炎综合征伴恶性紧张症
Intern Med. 2007;46(6):307-10. doi: 10.2169/internalmedicine.46.6179. Epub 2007 Mar 15.
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引用本文的文献

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Contemporary Encephalitis Lethargica: phenotype, laboratory findings and treatment outcomes.当代昏睡性脑炎:临床表现、实验室检查结果及治疗效果
J Neurol. 2009 Mar;256(3):396-404. doi: 10.1007/s00415-009-0074-4. Epub 2009 Mar 6.
2
Movement disorder emergencies.运动障碍急症
J Neurol. 2008 Aug;255 Suppl 4:2-13. doi: 10.1007/s00415-008-4002-9.