Clinical and electrophysiological analysis of limb-girdle muscular dystrophy:A geneological report
- VernacularTitle:肢带型肌营养不良家系的临床及电生理分析
- Author:
Yuegui CHEN
;
Tiebin YAN
;
Woliang YUAN
;
Jingfeng WANG
;
Ruqiong NIE
;
Enxiang TAO
;
Yingmei LIU
;
Yu MIN
;
Hailian YAN
- Publication Type:Journal Article
- Keywords:
Limb-girdle muscular dystrophy;
Clinical features;
Electrophysiology
- From:
Chinese Journal of Physical Medicine and Rehabilitation
2008;30(8):543-545
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical and electrophysiological features of one geneology with limbgirdle muscular dystrophy(LGMD). Methods Twenty-seven members of one family with limb-girdle muscular dystrophy(LGMD)were investigated.Fourteen of them were examined with electromyography(EMG)and their motor conduction velocities(MCV)and sensory conduction velocities(SCV)were measured.Among them,10 had no clinical manifestations,while 4 demonstrated symptoms and signs of LGMD. Results Three of the 4 patients had suffered from LGMD when young.They demonstrated the typical clinical features,including the progressive muscle weakness in the upper and lower extremities,positive Gower signs,duck gait,muscle atrophy distributed tO the proximal extremity,and no gastrocnemius hypertrophy.One subject presented atypical characteristics.The MCVs and SCVs of the 4 patients were normal,but neuropathic manifestations were found in the EMGS of 3 of them.and mixed neuropathic and myopathic manifestations were found in the EMG of the other.Conclusion LGMD patients in the same family can vary in their clinical characteristics.The longer the duration,the more severe the clinical features.Electrophysiological examination can reveal normal MCV and SCV but abnormal elctromyography.
