干燥综合征患者伴周围神经病变的临床电生理及病理特点  

Clinico-electrophysiological and pathological characteristics of neuropathy in Sj（o）gren＇s syndrome： a report of 5 cases

作　　者：张英爽[1] 万宇[1] 张斌[1] 董荣芳[2] 钟延丰[2] 廖琴[3] 樊东升[1] 

机构地区：[1]北京大学第三医院神经科,100191 [2]北京大学医学部病理学系 [3]北京积水潭医院神经科

出　　处：《中华医学杂志》2014年第11期855-858,共4页National Medical Journal of China

摘　　要：目的 总结原发性干燥综合征伴发周围神经病变患者的临床电生理及周围神经的病理特点.方法 回顾性分析2011年1月至2013年6月北京大学第三医院神经科5例原发性干燥综合征伴发周围神经病变患者的临床表现、电生理特点及腓肠神经和皮肤的活检病理特点.结果 5例患者均为女性,年龄20～75岁,周围神经病病程0.5～60个月,总病程12 ～240个月,周围神经病变模式：多发性单神经病1例,对称性感觉运动多神经病1例,感觉神经元神经病3例.5例患者大直径有髓神经纤维中重度减少,3例伴发髓鞘松解,但无洋葱球形成,无再生丛,2例束间血管呈纤维闭塞性血管炎.结论 干燥综合征患者周围神经病变表现为多发性单神经病、对称性感觉运动多神经病和感觉神经元神经病等多种形式,部分患者腓肠神经活检有典型血管炎表现,部分病理改变主要为有髓神经纤维减少,轴索变性,无再生丛;病生理机制可能有血管炎、缺血性损伤以及后根节神经元免疫炎性损害等.Objective To summarize the clinical features, electrophysiological and neuropathological characteristics of peripheral nerves in patients with primary SjCigren＇s syndrome （SS）. Methods We retrospectively analyzed the clinical, electrophysiological and neuropathological characteristics of 5 female SS patients with neuropathy complications undergoing electrophysiology and sural nerve biopsy at our department from January 2011 to June 2013. Results They had an age range of 20 -75 years. The course of peripheral neuropathy ranged 0.5 - 60 months and the whole course 12 - 240 months. pSS-associated neuropathy included multiple mononeuropathy （n = 1 ）, symmetrical axonal sensorimotor polyneuropathy （ n = 1 ） and sensory ganglioneuronopathy （ n = 3 ）. All biopsies showed varying degrees of myelinated fiber loss. Three biopsies had axonal degeneration associated with demyelinafion. However, there was no formation of onion bulb regeneration or plexus. Necrotizing vasculitis was diagnosed in 2 cases. Conclusion The manifestations of peripheral nerve n pSS include multiple mononeuropathy, axonal symmetric sensorimotor polyneuropathy and sensory gangioneuronopathy. Sural biopsy shows typical necrotizing vasculitis in some cases and myelinated fiber loss and axonal degeneration in others. The pathogenic mechanisms of neurological involvement in pSS remain unknown. However, vaseulitis, isehemic and immunological insults resulting in sensory gangioneuronopathy have been described.

关 键 词：干燥综合征 血管炎性周围神经病 感觉神经元神经病 腓肠神经活检 

分 类 号：R593.2[医药卫生—内科学]