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表现为急性播散性脑脊髓炎和假性脑瘤的亚急性硬化性全脑炎

Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis and pseudotumour cerebri.

作者信息

Panda Akhila Kumar, Mehta Vachan Jayant, Maheshwari Siddharth, Kar Sujit Kumar

机构信息

Department of Neurology, Institute of Human Behaviour & Allied Sciences IHBAS, Delhi, India.

出版信息

BMJ Case Rep. 2013 Aug 20;2013:bcr-2013-009432. doi: 10.1136/bcr-2013-009432.

Abstract

Subacute sclerosing panencephalitis (SSPE) is a chronic progressive encephalitis of childhood and young adults due to persistent measles virus infection. The usual age of onset is between 5 and 15 years. There are wide varieties of presentations of SSPE described in the literatures. Variable clinical presentations may lead to diagnostic dilemma and unnecessary investigations especially in developing countries, where the measles is quite endemic and vaccination status is not up to the mark because of poor literacy and socioeconomic status. Good clinical correlations, neuroimaging findings, EEG and cerebrospinal fluid (CSF) marker for SSPE yield the clue to diagnosis. This case illustrates a 13-year-old boy presented with short history of intellectual decline, headache, papilloedema, cranial nerve palsy, myoclonus with suggestive neuroimaging mimicking acute disseminated encephalomyelitis (ADEM) and pseudotumour cerebri. Subsequently he was diagnosed to be a case of SSPE on the basis of CSF and serum measles antibody titer.

摘要

亚急性硬化性全脑炎(SSPE)是一种发生于儿童和青年的慢性进行性脑炎,由麻疹病毒持续感染所致。通常发病年龄在5至15岁之间。文献中描述了SSPE的多种表现形式。临床表现的多样性可能导致诊断困境和不必要的检查,尤其是在发展中国家,那里麻疹相当流行,且由于文化水平低和社会经济地位差,疫苗接种情况不佳。SSPE良好的临床相关性、神经影像学表现、脑电图及脑脊液(CSF)标志物可为诊断提供线索。本病例为一名13岁男孩,以智力下降、头痛、视乳头水肿、颅神经麻痹、肌阵挛起病,病程较短,神经影像学表现提示急性播散性脑脊髓炎(ADEM)和假性脑瘤。随后根据脑脊液和血清麻疹抗体滴度诊断为SSPE病例。

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