老年人海绵状脑病一例报告及文献复习

Spongiform encephalopathies in the elderly: a case report of clinicopathological and literature review

目的 探讨老年期发病的海绵状脑病的临床特点并分析误诊原因。 方法 报告 1例79岁老年海绵状脑病的临床病理诊断过程 ,并复习国内报道的经病理学证实的 8例 6 0岁以上老年海绵状脑病患者的临床特征及脑电图、MRI资料。 结果 老年人海绵状脑病急性发病者病程短 ,但都有进行性痴呆、肌阵挛及各种不自主运动发作。 6例脑电图有典型的周期性三相波 ,1例MRI发现双侧基底节区T2 高信号。 3例初诊为脑血管病 ,1例为单疱性脑炎。病理上有典型的空泡和海绵状改变。 结论 急性发病的老年人海绵状脑病容易误诊为脑血管病 ;但根据患者典型的临床表现 ,结合动态脑电图特征 ,必要时行脑活检 ,可尽早诊断 。

Objective To explore the clinical features of spongiform encephalopathies occurred in the elderly and causes of misdiagnosis. Methods Clinicopathological diagnosis of a 79 year old man with acute spongiform encephalopathy was reported. Clinical, EEG and MRI characteristics of eight old patients with spongiform encephalopathy in China confirmed by neuropathology were reviewed. Results Elder patients with spongiform encephalopathies had an acute onset and a rapid developing course, and all patients presented with progressive dementia accompanied by various clinical manisfestations of extrapyramidal diseases. EEG recordings with typical periodic sharp wave complexes (PSP) was shown in six cases, and abnormal T2 signal in both basal ganglia by MRI in one case. Three cases were misdiagnosed as cerebral vascular disease and one cse as encephalitis herpes cimplex. Conclusions Spongiform encephalopathy in the elderly should be paid more attention to since their early clinical symptoms are similar to that of the acute cerebral vascular diseases. It is suggested that early diagnosis of spongiform encephalopathy could be made basing on the combination of the typical clinical characteristics and dynamic EEG findings, and brain biopsy if necessary, to avoid nosocomial spreading.