摘要
目的分析脱髓鞘N4（HMSN1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HMSN1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MNCV35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±9．141m／s和（30．07±12．19）m／s。未检出不同神经SNCV50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623％；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HMSN1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。
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 treatment 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） rn/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 ofneurogenic 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.
出处
《中华神经医学杂志》
CAS
CSCD
北大核心
2013年第10期1032-1034,共3页
Chinese Journal of Neuromedicine
关键词
腓骨肌萎缩症
脱髓鞘型
神经传导速度
肌电图
神经电生理
Charcot-Mafie-Tooth disease
Hereditary motor and sensory neuropathy type 1
Nerve conduction velocity
Electromyography
Neurophysiology
