【摘要】 目的探讨复发性吉兰-巴雷综合征(RGBS)临床和病理特点。方法收集山东大学齐鲁医院神经内科1999-2016年确诊的RGBS患者,分析其临床表现、神经电生理和腓肠神经活检病理特点。结果 19例RGBS患者共发作41例次,两次发作间最长间隔27年,最短5个月,平均41个月。起病年龄15~69岁,平均44岁,男女比例为8∶11。41例次发作中有26例次发病前有明确感染史,2例次复发与静脉注射神经节苷脂相关。RGBS患者在临床表现、脑脊液及神经电生理改变方面与非RGBS无明显区别。3例患者行腓肠神经活检,主要病理改变为有髓纤维数量重度减少,可见洋葱皮样结构。结论 RGBS患者临床特点与非RGBS相似,但症状、体征与初次发作比较有明显加重倾向。以轴索损害为主的发作容易累及呼吸肌,脱髓鞘型比轴索型更容易复发。RGBS患者中腓肠神经活检洋葱皮样结构常见。更多还原

【Abstract】 Objective To investigate the clinical and pathological features of recurrent Guillain-Barré syndrome(RGBS). Methods The clinical data were obtained from the medical records of RGBS patients during 1999 to 2016 in the Department of Neurology, Qilu Hospital of Shandong University. The clinical manifestation, electrophysiology and sural nerve biopsy were collected and summarized retrospectively. Results A total of 19 RGBS patients including 8 males and 11 females, who had a total of 41 attack episodes, were identified. The interval between attacks ranged from 5 months to 27 years, averaging 41 months. The mean age at episode was 44 years(range 15~69 years). Preceding infections were reported 26 times. Another 2 patients had treatment with ganglioside prior to one episode. The RGBS patients showed similar clinical features, cerebrospinal fluid findings, and electrophysiologic characteristics compared to GBS patients. A total of 3 sural nerve biopsy cases revealed marked loss of myelinated fibers. Onion-bulb formations were presented in 2 cases. No inflammatory cells were found. Conclusion For RGBS patients, the neurological symptoms and electrophysiologic characteristics in subsequent episodes are often similar to that of GBS patients. However, the symptoms severity and the nature of preceding infections in both patients are varied.更多还原