摘要
目的 探讨Lewis-Sumner综合征的临床、病理、电生理特点及治疗疗效.方法 回顾北京大学第三医院神经科2008年5月至2014年8月诊治的9例Lewis-Sumner综合征临床表现、电生理特点、腓肠神经活检病理特点、治疗及随访情况.结果 9例患者中,男7例,女2例,平均发病年龄29（18 ～64）岁,治疗后随访6～48个月.9例患者均为不对称起病,有8例以远端肢体受累发病.5例患者发病时有感觉和运动障碍,3例只有运动症状,仅有1例以感觉症状起病.病情演变主要以多灶性分布和对称性分布两种形式.神经电生理提示非嵌压部位的运动神经传导阻滞,受累的神经以正中神经和尺神经为主.腓肠神经病理活检仅见到神经轻度脱髓鞘性变性,无炎性细胞浸润现象.经免疫治疗后患者症状都有不同程度的改善,预后较好.结论 Lewis-Sumner综合征多以上肢远端不对称起病,以无力和麻木为主要表现,最常受累的神经为正中神经和尺神经,电生理检测可见有运动神经的传导阻滞,病理特点以轻度脱髓鞘病变为主.免疫治疗有效.
Objective To investigate the clinical characteristics,electrophysiological findings and treatment response of Lewis-Sumner syndrome （LSS）.Methods Data of nine patients with LSS,who were diagnosed and treated from May 2008 to August 2014 in Department of Neurology,Peking University Third Hospital,were analyzed retrospectively,including clinical features,electrophysiological studies,pathological characteristics,therapy and follow up.Results The nine cases included seven males and two females,with the average age being 29 years old （18-64 years old）.The follow-ups after treatment were between 6 months to 48 months.All 9 cases were asymmetrical onset,and the distal part of limbs was initially affected in 8 cases.Five cases presented with the initial symptom of sensorimotor,3 cases with purely motor and only 1 case with sensory.The disease developed two forms：multifocal distribution and symmetrical distribution.Electrophysiological studies demonstrated conduction blocks on non-entrapment sites,involving mainly median nerve and ulnar nerve.Sural nerve biopsy presented mild demyelination without inflammatory infiltration.The symptoms of patients had some degrees of improvement after immunotherapy,and the prognosis was favorable.Conclusions Lewis-Sumner syndrome presents the initial symptoms of asymmetrical sensorimotor neuropathy mostly affecting the upper extremities.Median nerve and ulnar nerve are the most common involvements.Electrophysiological studies demonstrate motor nerve conduction block.The major finding of pathology is mild demyelinated.The patients have a positive response to immunotherapy.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2016年第11期859-862,共4页
National Medical Journal of China
