慢性炎性脱髓鞘性多发性神经病与腓骨肌萎缩症-I型的临床及神经电生理比较

目的比较慢性炎性脱髓鞘性多发性神经病(chronic inflammatory demyelinating polyneuropathy,CIDP)与腓骨肌萎缩症-I型(type-I Charcot Marie Tooth disease,CMT-I)的临床及神经电生理特点,以指导两者的鉴别诊断。方法纳入CIDP患者31例、CMT-I患者28例,收集其一般临床资料并对两组患者进行神经电生理检测,比较两组患者的临床特点及电生理指标,并对电生理严重程度与临床症状严重程度进行相关性分析。结果 CIDP与CMT-I两组患者起病部位、主观感觉障碍、足部畸形、脑脊液蛋白比较有统计学差异(P0.05)。运动末梢潜伏期(distal motor latency,DML)、运动传导速度(motor conduction velocity,MCV)、感觉传导速度(sensory conduction velocity,SCV)、传导阻滞/波形离散、下肢神经继发性轴索变性具有统计学差异(P0.05)。失神经电位、MUAP形态异常、募集减少具有统计学差异(P0.05)。CIDP临床症状严重程度与电生理严重程度有相关性(r=0.84,P0.05);而CMT-I临床症状严重程度与电生理严重程度分离,不具有相关性(r=0.27,P0.05)。结论综合分析临床特点、神经传导、针极肌电图、临床与电生理严重程度的相关性可以对CIDP与CMT-1进行鉴别诊断。

Comparison of clinical manifestations and electrophysiological features in patients with chronic inflamma-tory demyelinating polyneuropathy and Type-I Charcot Marie Tooth Disease

Objectives To compare clinical manifestations and electrophysiological features in patients with chron?ic inflammatory demyelinating polyneuropathy (CIDP) and Type-I Charcot Marie Tooth Disease (CMT-I) for guiding dif?ferential diagnosis. Methods Data including clinical manifestations and electrophysiological indexes was collected from thirty-one CIDP cases and 28 CMT-I cases. Correlation analysis was used to assess the association of the severity of electrophysiology with the severity of clinical symptoms. Results There were statistically significant differences in onset site, sensory dysfunction, foot deformity and cerebrospinal fluid protein between these two groups (P<0.05). There were significant differences in indexes of nerve conduction and needle electromyography between these two groups (P<0.05). The severity of clinical symptoms was not related with the severity of electrophysiology in CMT-I group (r=0.27,P>0.05). Conclusions Differential diagnoses of CIDP and CMT-I can be made based on clinical manifestations and electro?physiological features.