Neurophysiological performance of Charcot-Marie-Tooth disease (hereditary motor and sensory neuropathy type 1)
10.3760/cma.j.issn.1671-8925.2013.10.013
- VernacularTitle:脱髓鞘型腓骨肌萎缩症的神经电生理表现
- Author:
Rao WANG
1
;
Yin ZHU
;
Zhuo YANG
;
Li-Hua WANG
;
Yun YANG
;
Zhen-Zhen ZHANG
Author Information
1. 北京积水潭医院肌电图室
- Keywords:
Charcot-Marie-Tooth disease;
Hereditary motor and sensory neuropathy type 1;
Nerve conduction velocity;
Electromyography;
Neurophysiology
- From:
Chinese Journal of Neuromedicine
2013;12(10):1032-1034
- CountryChina
- Language:Chinese
-
Abstract:
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.
