中国Fisher综合征126例临床特点分析

Clinical features of Fisher syndrome in 126 Chinese patients

目的了解我国Fisher综合征患者的临床特点。方法检索维普电子期刊全文数据库于1989-2010年收录的Fisher综合征病例报告文献,并分析所报道病例的临床特点。结果共纳入68个研究,包括126例患者,其中男性78例,女性48例,发病年龄最小4岁,最大78岁,平均发病年龄(40.0±19.4)岁。82例(65.1%)有前驱感染史,101例(80.2%)同时出现共济失调、腱反射减弱或消失及眼肌麻痹三联征;病程最短13d,最长90 d,平均病程(43.0±21.0)d,121例患者进行了脑脊液检查,其中101例(83.5%)出现脑脊液蛋白细胞分离现象。男性及女性临床特点无统计学差异(P>0.05)。79例行电生理检查,其中71例(89.9%)患者合并周围神经损害。结论中国Fisher综合征以中青年常见,男性多见,多数患者有前驱感染史,多出现共济失调、腱反射减弱或消失、眼肌麻痹等三联征,大部分患者出现脑脊液蛋白细胞分离现象,电生理提示周围神经和神经根损害。

Objective To study the clinical features of Fisher syndrome in China. Methods Cases reports of Fisher syndrome from 1989 to 2010 were retrieved from the Weipu database by computer using the key words of ＂Fisher syndrome＂, ＂MillevFisher syndrome＂ and ＂Guillain-Barre syndrome ＋ variation＂. Clinical features of reported patients with Fisher syndrome were analyzed. Results Sixty-eight reports were enrolled. Totally, 126 patients including 78 males and 48 females were included in the study. The mean onset age was 40.0±19.4 years （range 4-78 years）. Antecedent infection was found in 82 （65.1%） patients. 101 （80.2%） patients were characterized by the clinical traid of ophthalmoplegia, ataxia and areflex. The average course of disease was （43.0 ±21.0） days （range 13 ~90 days）. Albuminocytolgoic dissociation was showed in 101 （83.5%） of the 121 patients who had adopted cerebrospinal fluid examination. There were no significant differences of clinical features between males and females （P〉 0.05）. Electrophysiology studies demonstrated peripheral nervous damage in 71 （89.9%） of the 79 patients. Conclusions In China, Fisher syndrome was mainly seen in the youth and middle-aged, with male predominance. Preceding infection was found in most patients with Fisher syndrome in China. The majority presented with the clinical traid and albuminocytolgoic dissociation phenomena. Peripheral nerve and nerve root lesions were presented on electrophysiology.