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神经阻滞剂恶性综合征作为亚急性硬化性全脑炎的表现特征。

Neuroleptic malignant syndrome as a presenting feature of subacute sclerosing panencephalitis.

机构信息

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.

出版信息

J Neurovirol. 2018 Feb;24(1):128-131. doi: 10.1007/s13365-017-0602-4. Epub 2017 Dec 14.

Abstract

Subacute sclerosing panencephalitis (SSPE) is a slowly progressive degenerative disorder caused by measles virus. It is characterised by typical clinical and electrophysiological features in the form of slow myoclonic jerks, with progressive cognitive impairment, visual symptoms, and periodic complexes on EEG, with raised titres of anti-measles antibodies in CSF and serum. Atypical presentations of SSPE have been reported including brainstem involvement, ADEM-like presentation, acute encephalitis, and cerebellar ataxia. Presentation with predominant extrapyramidal features is uncommon. We describe a case of SSPE presenting with extensive rigidity with highly elevated CPK values, mimicking neuroleptic malignant syndrome (NMS) which was most probably due to central dopaminergic blockade induced by the disease process. To our knowledge, this is the first case of SSPE presenting with a NMS-like syndrome.

摘要

亚急性硬化性全脑炎(SSPE)是一种由麻疹病毒引起的缓慢进行性退行性疾病。其特征是出现典型的临床和电生理特征,表现为缓慢肌阵挛性抽搐,伴有进行性认知障碍、视觉症状和脑电图上的周期性复合波,脑脊液和血清中抗麻疹抗体滴度升高。也有报道称 SSPE 存在不典型表现,包括脑干受累、类似急性播散性脑脊髓炎的表现、急性脑炎和小脑共济失调。以锥体外系表现为主的表现并不常见。我们描述了一例以广泛僵硬为主要表现的 SSPE 患者,其肌酸磷酸激酶(CPK)值极高,类似于神经阻滞剂恶性综合征(NMS),这很可能是由于疾病过程导致的中枢多巴胺能阻断所致。据我们所知,这是首例以 NMS 样综合征为表现的 SSPE 病例。

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