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亚急性硬化性全脑炎的非典型表现:4例临床研究

Atypical presentations of SSPE: a clinical study in four cases.

作者信息

Demir Ercan, Aksoy Ayşe, Anlar Banu, Sönmez Fatma Müjgan

机构信息

Department of Pediatric Neurology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey.

出版信息

Turk J Pediatr. 2007 Jul-Sep;49(3):295-300.

Abstract

Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal disease of the central nervous system caused by a persistent measles virus. It is clinically characterized by insidious onset of intellectual deterioration and behavioral changes followed by myoclonias and eventually complete neurologic deterioration. The diagnosis is based on characteristic clinical features, periodic electroencephalography (EEG) complexes of high slow waves and increased antibody titer against measles in cerebrospinal fluid. Here, we report four SSPE cases, two of whom manifested with hemiparesis; in the third and fourth cases, cerebellar ataxia and acute encephalopathy with focal seizures were the presenting symptoms at the onset of disease, respectively. The typical periodic EEG complexes in our patients led to the diagnosis of SSPE. Our findings show that SSPE should be considered in the differential diagnosis of hemiparesis, cerebellar ataxia and acute encephalopathy, and highlight the diagnostic significance of EEG in unidentified cases.

摘要

亚急性硬化性全脑炎(SSPE)是一种由持续性麻疹病毒引起的中枢神经系统进行性致命疾病。其临床特征为隐匿起病的智力衰退和行为改变,随后出现肌阵挛,最终完全神经功能衰退。诊断基于特征性临床特征、周期性脑电图(EEG)高慢波复合波以及脑脊液中抗麻疹抗体滴度升高。在此,我们报告4例SSPE病例,其中2例表现为偏瘫;第3例和第4例分别以小脑共济失调和伴有局灶性癫痫发作的急性脑病为疾病发作时的首发症状。我们患者典型的周期性EEG复合波导致了SSPE的诊断。我们的研究结果表明,在偏瘫、小脑共济失调和急性脑病的鉴别诊断中应考虑SSPE,并强调EEG在不明病例中的诊断意义。

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