摘要
目的 总结6例以周围神经病为主要表现的显微镜下多血管炎（MPA）的临床和病理特点,探索该病微血管损伤特点.方法 收集6例经过临床症状、抗体检查、病理证实的MPA患者详细的临床资料,进行神经电生理检查及腓肠神经活体组织检查.2例正常神经作为形态学对照.结果 6例患者均出现感觉神经损害为主的非对称性多发单神经病.电生理检查显示感觉神经诱发电位和运动神经复合肌肉动作电位波幅下降为主要改变.病理检查发现有髓神经纤维变性,单核细胞和淋巴细胞浸润为主偶伴嗜酸细胞浸润的血管炎性改变.结论 MPA的周围神经损害表现为以感觉神经损害为主的非对称性神经病,电生理改变以多发单神经的轴索损害为主,病理检查具有急性轴索损害以及血管炎改变.
Objective To investigate the clinical and pathologic features in patients of microscopic polyangiitis （MPA） with peripheral nerve abnormality. Methods We collected clinical data of 6 patients with MPA that was confirmed by immunological, pathological and clinical findings. Eleetroneurophysiologic examinations and sural nerve biopsies were performed in these patients. Two normal controls were included in these studies. Results All of 6 patients developed asymmetric polyneuropathies. Eleetrodiagnostic studies showed reduced amplitudes of the sensory nerve action potential and compound motor action potential with mild impaired conductive velocities. The patients presented vasculitis changes with cell infiltration by monoeytes and lymphocytes. Sural nerve biopsies found loss of myelinated fibers in all of the patients indicating axonal degeneration. Some of them associated with regeneration clusters of myelinated fibers. Conclusions Asymmetric neuropathy with sensory nerve injuries is the mainfeature in the MPA associated polyneuropathy. Eleetrodiagnostie examination indicated axonal lesions in mononeuritis multiplex. Sural nerve biopsies confirmed the active axonal lesions and vasculitis.
出处
《中华神经科杂志》
CAS
CSCD
北大核心
2014年第5期324-326,共3页
Chinese Journal of Neurology
