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病例报告:以局灶性肌阵挛伴纹状体病变为表现的亚急性硬化性全脑炎

Case Report: Focal Myoclonus with a Striatal Lesion as a Presentation of Subacute Sclerosing Panencephalitis.

作者信息

Kalita Jayantee, Chaudhary Sarvesh K, Kumar Bishwanath, Jadhav Mahesh

机构信息

Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Department of Neurology, Adhikari Neurocentre, Patna, India.

出版信息

Am J Trop Med Hyg. 2022 May 9;106(6):1729-31. doi: 10.4269/ajtmh.22-0046.

DOI:10.4269/ajtmh.22-0046
PMID:35533695
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9209915/
Abstract

Adult-onset subacute sclerosing panencephalitis (SSPE) is rare, and focal myoclonus as a presenting feature poses a diagnostic dilemma. We report an adult SSPE patient with unusual clinical and radiological features. A 20-year-old girl had jerky neck movement 9 months earlier, which progressed to left hemimyoclonus in 2 months and generalized frequent myoclonus and fall at 4 months. By 6 months, she was bedbound. On examination, her Mini-Mental State Examination score was 10, and patchy retinitis was observed around the macula. Magnetic resonance imaging revealed corpus striatal involvement and electroencephalography showed periodic discharges. Measles cerebrospinal fluid/serum immunoglobulin G index was 3.3 (normal < 1.3), confirming the diagnosis of SSPE. SSPE should also be considered in adults having focal myoclonus with corpus striatal lesion. EEG is helpful in the diagnosis.

摘要

成人起病的亚急性硬化性全脑炎(SSPE)较为罕见,以局灶性肌阵挛为首发表现会带来诊断难题。我们报告一例具有不寻常临床和影像学特征的成人SSPE患者。一名20岁女孩9个月前出现颈部抽搐性运动,2个月后进展为左侧偏身肌阵挛,4个月时出现全身性频繁肌阵挛和跌倒。到6个月时,她卧床不起。检查时,她的简易精神状态检查表评分为10分,黄斑周围可见斑片状视网膜病变。磁共振成像显示纹状体受累,脑电图显示周期性放电。麻疹脑脊液/血清免疫球蛋白G指数为3.3(正常<1.3),确诊为SSPE。对于有局灶性肌阵挛伴纹状体病变的成人,也应考虑SSPE。脑电图有助于诊断。

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