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先天性副肌强直是显性遗传性疾病,病程呈非进行性.病人遇冷后出现肌肉僵硬,肌肉活动时更为明显.寒冷尚可产生严重的肌无力,且缓解很缓慢,常可持续几小时.主要为手和面部受累.已往药物治疗无效,因而病人竭力使自己保持温暖.近来研究认为:副肌强直病人肌膜的钠通道机能失调.遇冷期间,副肌强直病人的肌膜静息膜电位降低.Lehmann-Horn等人描记三例先天性副肌强直病人肋间肌纤维细胞内的电活动.当温度从37℃降至27℃时静息膜电位从-80mv降至-40mv.后来又
Congenital paraneoplastic ankylosis is a dominant genetic disease, the course was non-progressive. Patients with muscle stiffness after the cold, muscle activity more pronounced. Cold can still produce severe muscle weakness, and the relief is very slow, often sustainable A few hours mainly hand and face involvement in the past drug treatment is invalid, so the patient tried to keep himself warm recently research that muscle dysfunction in the sarcolemma of accessory muscle myotonia patients, Resting membrane potential was reduced.Lehmann-Horn et al. Traced the electrical activity within the intercostal muscle cells of three patients with congenital paralothus. The resting membrane potential decreased from -80 mv to -40 mv as the temperature dropped from 37 ° C to 27 ° C And later