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无感觉丧失的吉兰-巴雷综合征(急性运动性神经病)。具有特定临床、电诊断和实验室特征的一个亚组。荷兰吉兰-巴雷研究组。

Guillain-Barré syndrome without sensory loss (acute motor neuropathy). A subgroup with specific clinical, electrodiagnostic and laboratory features. Dutch Guillain-Barré Study Group.

作者信息

Visser L H, Van der Meché F G, Van Doorn P A, Meulstee J, Jacobs B C, Oomes P G, Kleyweg R P, Meulstee J

机构信息

Department of Neurology, University Hospital Dijkzigt, The Netherlands.

出版信息

Brain. 1995 Aug;118 ( Pt 4):841-7. doi: 10.1093/brain/118.4.841.

Abstract

We analysed data obtained from 27 out of a group of 147 patients with Guillain-Barré syndrome, who did not have sensory loss during a follow-up period of 6 months (motor Guillain-Barré syndrome). These patients had a distinctive clinical pattern compared with the other 120 Guillain-Barré syndrome patients. The clinical course was marked by a more rapid onset of weakness (3.9 versus 6.1 days, P = 0.002), an earlier nadir (6.3 versus 9.1 days, P < 0.001), an initially predominant distal weakness (67% versus 27%, P < 0.001), sparing of the cranial nerves (26% versus 68%, P < 0.001) and the disease was more often preceded by a gastro-intestinal illness (41% versus 13%, P = 0.001) often caused by a Campylobacter jejuni infection (67% versus 28% in the other Guillain-Barré syndrome patients, P < 0.001). High titres of anti-GM1 antibodies were also significantly more common in motor Guillain-Barré syndrome patients (42% versus 5%, P < 0.001). Electromyographic data of the motor Guillain-Barré syndrome patients at nadir revealed little or no evidence for demyelination. Abundant denervation activity was present in half of the patients. The response to immune globulin treatment was good but with plasma exchange significantly fewer motor Guillain-Barré syndrome patients reached the stage of independent locomotion after a follow-up period of 6 months especially if the acute motor neuropathy occurred after a C.jejuni infection.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

我们分析了147例吉兰-巴雷综合征患者中27例的数据,这些患者在6个月的随访期内没有感觉丧失(运动型吉兰-巴雷综合征)。与其他120例吉兰-巴雷综合征患者相比,这些患者有独特的临床模式。临床病程的特点是肌无力起病更快(3.9天对6.1天,P = 0.002),最低点出现更早(6.3天对9.1天,P < 0.001),最初以远端肌无力为主(67%对27%,P < 0.001),颅神经受累较少(26%对68%,P < 0.001),且该疾病更常先有胃肠道疾病(41%对13%,P = 0.001),常由空肠弯曲菌感染引起(在其他吉兰-巴雷综合征患者中为67%对28%,P < 0.001)。高滴度抗GM1抗体在运动型吉兰-巴雷综合征患者中也明显更常见(42%对5%,P < 0.001)。运动型吉兰-巴雷综合征患者最低点时的肌电图数据显示几乎没有脱髓鞘证据。一半患者存在大量失神经活动。免疫球蛋白治疗反应良好,但血浆置换治疗后,在6个月的随访期后,尤其是急性运动神经病由空肠弯曲菌感染引起时,能达到独立行走阶段的运动型吉兰-巴雷综合征患者明显更少。(摘要截选至250字)

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