CAJ | 학술논문

目的分析脱髓鞘型(HMSN 1型)腓骨肌萎缩症的神经电生理表现,为该疾病的临床诊断治疗提供检查依据.方法选取自2011年1月至2012年12月北京积水潭医院收治的62例HMSN 1型腓骨肌萎缩症患者,行神经传导速度与肌电图检查,神经传导速度包括运动神经传导速度(MNCV)与感觉神经传导速度(SNCV),对检查结果进行分析.结果未检出不同神经MNCV 35例,占56.5％；胫神经、腓总神经与正中神经的平均MNCV检测结果分别是(23.48±9.60)m/s、(26.35±9.14) m/s和(30.07±12.19) m/s.未检出不同神经SNCV 50例,占80.6％；胫神经与正中神经的平均SNCV检测结果分别是(28.06±10.32) m/s和(31.20±12.05) m/s.肌电图检查共175块肌肉,其中109块肌肉判定为神经源性损害,占62.3％; 14块肌肉判定为可疑神经源性损害,占8.0％；52块肌肉正常,占29.7％.62例中有神经源性损害者53例,占85.5％,其中远端肌肉有神经源性损害者47例,近端肌肉有神经源性损害者11例.结论 HMSN 1型腓骨肌萎缩症患者的神经电生理表现随着患者的年龄、病程和检查部位不同而变化.
목적 분석탈수초형(HMSN 1형)비골기위축증적신경전생리표현,위해질병적림상진단치료제공검사의거.방법 선취자2011년1월지2012년12월북경적수담의원수치적62례HMSN 1형비골기위축증환자,행신경전도속도여기전도검사,신경전도속도포괄운동신경전도속도(MNCV)여감각신경전도속도(SNCV),대검사결과진행분석.결과 미검출불동신경MNCV 35례,점56.5％；경신경、비총신경여정중신경적평균MNCV검측결과분별시(23.48±9.60)m/s、(26.35±9.14) m/s화(30.07±12.19) m/s.미검출불동신경SNCV 50례,점80.6％；경신경여정중신경적평균SNCV검측결과분별시(28.06±10.32) m/s화(31.20±12.05) m/s.기전도검사공175괴기육,기중109괴기육판정위신경원성손해,점62.3％; 14괴기육판정위가의신경원성손해,점8.0％；52괴기육정상,점29.7％.62례중유신경원성손해자53례,점85.5％,기중원단기육유신경원성손해자47례,근단기육유신경원성손해자11례.결론 HMSN 1형비골기위축증환자적신경전생리표현수착환자적년령、병정화검사부위불동이변화.
Objective To find out the neurophysiological performance of Charcot-Marie-Tooth disease (CMT) of hereditary motor and sensory neuropathy (HMSN) type 1,and provide examination basis for clinical diagnosis and Ureatment of the disease.Methods Sixty-two patients with CMT of HMSN type 1,admitted to our hospital from January 2011 to December 2012 were chosen in our study; the nerve conduction velocity,including motor nerve conduction velocity (MNCV) and sensory nerve conduction velocity (SNCV),and electromyogram (EMG) of these patients were examined.All the test results were analyzed statistically.Results MNCV in different nerves could not be detected in 35 patients,counting for 56.5％; removing the cases that MNCV not being elicited,average test results of tibial nerve,common peroneal nerve and median nerve MNCV were (23.48±9.60) m/s,(26.35±9.14) m/s and (30.07±12.19) m/s.SNCV in different nerves could not be detected in 50 patients,counting for 80.6％; removing the cases that SNCV not being elicited,average test results of common peroneal nerve and median nerve MNCV were (28.06±10.32) m/s and (31.20±12.05) m/s.Totally,175 muscles were inspected by EMG; among them,109 neurogenic muscles were determined,counting for 62.3％; 14 muscles were diagnosed as having suspect neurogenic damage,counting for 8.0％; 52 normal muscles were noted,counting for 29.7 ％.There were 53 of neurogenic damages in 62 cases,counting for 85.5％; among them,the distal neurogenic muscles damages were in 47 cases; while proximal neurogenic muscle damages were in 11 cases.Conclusion The neurophysiological manifestations of CMT patients of HMSN type 1 are different and changes following the patient's age,duration and examination site.