副肿瘤性周围神经病的临床、电生理及病理改变特点  

The clinical, electrophysiological and nerve pathological features of paraneoplastic peripheral neuropathy

作　　者：黎思娴 吕鹤[1] 贾志荣[1] 石昕[1] 梁为[1] 郝洪军[1] 左越焕[1] 

机构地区：[1]北京大学第一医院神经内科,100034

出　　处：《临床神经病学杂志》2018年第1期6-9,共4页Journal of Clinical Neurology

摘　　要：目的探讨副肿瘤性周围神经病(PPN)的临床、电生理及病理改变特点。方法回顾性分析4例PPN患者的临床资料。结果 4例患者均以疼痛、麻木等感觉症状起病,其中3例首先累及双下肢,3例双下肢不对称受累,3例累及植物神经,2例累及颅神经,所有患者周围神经症状均由远端向近端进行性发展。4例患者均出现感觉神经传导异常,其中3例以轴索损害为主,1例以脱髓鞘损害为主;1例伴有运动神经传导异常,以脱髓鞘损害为主。4例行腓肠神经活检的患者中,3例为活动性轴索改变,1例为轴索和髓鞘混合性改变,4例均无小血管周围炎性改变。结论 PPN多为不对称分布的感觉神经病,进行性发展,颅神经和植物神经受累常见。PPN的电生理以感觉神经传导异常为主、轴索损害较重。活动性轴索变性是PPN的主要病理改变,极少伴有小血管周围炎。Objective To explore the clinical, electrophysiological and nerve pathological features of paraneoplastic peripheral neuropathy （PPN）. Methods The clinical data of 4 PPN patients were analyzed retrospectively. Results Onset symptom of 4 patients was sensory symptoms such as pain and numbness. Both lower extremities involved was in 3 cases, asymmetrical involvement of both lower extremities was in 3 cases, autonomic nervous involved was in 3 cases, cranial nerve involved was in 2 cases. All the patients developed from distally to proximally. The sensory nerve conduction abnormal was in 4 cases, axonal injury was in 3 cases, demyelinating injury was in 1 case; motor nerve conduction abnormal was in 1 case, mainly with demyelinating injury. Sural nerve biopsy was checked in 4 cases, actively of axonal injury was in 3 cases, axonal and myelin sheath as maxed injury was in 1 case. None of them had small vessels vasculitis. Conclusions PPN is mainly asymmetrical sensory neuropathy, and keep progressive development. Cranial nerves and autonomic nerve involved are common. Sensory nerve conduction abnormal is common in electrophysiology examination of PPN, and axonal injury is serious. Actvely axonal injury is major pathological change of PPN, and rarely accompanied with small vaseulitis.

关 键 词：副肿瘤性周围神经病 副肿瘤相关抗体 感觉神经病 轴索性周围神经病 

分 类 号：R745[医药卫生—神经病学与精神病学]