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目的 研究慢性格林 巴利综合征 (CIDP)的临床、电生理及病理特征 ,探讨腓肠神经活检的诊断价值。方法 总结 4 9例CIDP患者的临床表现 ,病程特点 ,腰穿脑脊液 (CSF)检查、肌电图检查以及 2 1例患者的腓肠神经活检病理结果。结果 本组 4 9例CIDP患者大多无明显前驱因素 ,临床表现为对称性肢体运动和感觉障碍 ,少数 (16例 )可伴颅神经损害 ;脑脊液蛋白量波动较大 ,单次腰穿 2 4例可见蛋白细胞分离 ;大多数肌电图 (39例 / 4 6例 )提示有脱髓鞘损害 ,少数有轴索损害 (15例 / 4 6例 )。 2 1例患者行腓肠神经病理检查 ,显示明显脱髓鞘病灶 17例 ,轴索变性 5例 ,洋葱头样改变 5例 ,炎性细胞浸润 11例。结论 电生理和病理检查均提示CIDP是以神经脱髓鞘改变为主 ,对临床表现不典型者腓肠神经活检有较大的诊断价值。
Objective To investigate the clinical, electrophysiological and pathological features of chronic Guillain-Barre syndrome (CIDP) and to explore the diagnostic value of sural nerve biopsy. Methods The clinical manifestations, course of disease, cerebrospinal fluid (CSF) examination and EMG examination of 49 CIDP patients were summarized. The pathological results of sural nerve biopsy in 21 patients were summarized. Results Most of the 49 cases of CIDP patients had no obvious predisposing factors. The clinical manifestations were symmetrical limb movement and sensory disturbance. A small number of 16 patients could be associated with cranial nerve damage. The cerebrospinal fluid protein content fluctuated greatly. A single lumbar puncture was performed in 24 patients Most of the EMG (39 cases / 46 cases) showed demyelination and few axonal injuries (15 cases / 46 cases). Twenty-one patients underwent sural nerve pathology examination, which showed 17 cases of obvious demyelinating lesions, 5 cases of axonal degeneration, 5 cases of onion head-like changes and 11 cases of inflammatory cell infiltration. Conclusions Both electrophysiological and pathological findings suggest that CIDP is predominantly demyelinated, and that it is of great diagnostic value for sural biopsy of atypical clinical manifestations.