慢性炎性脱鞘性多神经根神经病的电生理学和病理学研究

Clinical electrophysiological and pathological study on chronic inflammatory demyelinating polyradiculoneuopathy

目的 研究慢性炎性脱鞘性多神经根神经病 (CIDP)的临床电生理学和病理学特点。方法 采用CounterpointMKⅡEMG肌电图仪对 16例CIDP患者进行运动神经传导速度 (MCV)、感觉神经传导速度 (SCV)、F波和常规肌电图检查 ;所有患者均取腓肠神经交通枝制作光镜和电镜切片进行病理学研究。结果 电生理结果显示受累神经以脱鞘为主 ,表现为运动神经传导阻滞 (CB)、短暂性波形离散 (TD)、F波潜伏期延长和传导速度减慢。CB和TD为CIDP神经脱鞘的重要电生理改变。病理学主要表现为神经脱鞘、雪旺细胞变性、少量单核淋巴细胞浸润 ,可以伴有神经纤维的再生和“洋葱球”样结构的形成。部分病例发现伴有轴索损害。结论 髓鞘脱失是CIDP主要的电生理学特点 ,但是对于CIDP的电生理诊断应该采用更为严格的标准。雪旺细胞变性有时可以出现在髓鞘和轴索的改变之前 。

Objective In the present studies, clinical electrophysiological and pathological features of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were investigated in detail.Methods Electrophysiological studies were performed on 16 patients with CIDP by using Counterpoint MKⅡEMG machine. The items included motor conduction velocity (MCV), sensory conduction velocity (SCV), F reflex. All patients underwent sural nerve biopsy at the midcalf. Standard techniques were used to evaluate the evaluated the specimens by light and electron microscopy.Results Eelectrophysiological results showed the evidence of demyelination, including motor nerve conduction block (CB), temporal dispersion (TD), abnormal F waves, prolonged distal latencies and conduction slowing. Both CB and TD could be considered as major features. Biopsy detection of patients with CIDP was characterized by active demyelination, Schwann cell degeneration,sparse mononuclear cell infiltrates together with remyelinating fibers and “onion bulb” formations. Axonal changes with segmental demyelination could be found sometimes. Conclusion Demyelination was main characteristic changes of the peripheral nerves in CIDP, the strict diagnostic criteria should be applied. Degeneration of Schwann cells, which may happen before demyelination and axonal changes at times, may be another feature of CIDP.