强直性肌营养不良——5个家系21例及散发3例的分析  

MYOTONlC DYSTROPHY: A STUDY OF 24 CASES

作　　者：侯辉光 陈达仁 

机构地区：[1]第二军医大学长海医院神经科 [2]第二军医大学长征医院神经科

出　　处：《上海医学》1983年第3期149-152,共4页Shanghai Medical Journal

摘　　要：报告强直性肌营养不良24例,其中21例分布在5个家系,3例散发;起病年龄2例在15岁以下,其余22例在20~28岁之间。临床表现除肌萎缩、肌强直外,尚有多系统病变的症状。肌电图检查有强直性电位。4例肌肉活检示不同程度的变性、萎缩,但神经末梢和运动终板完好,似不支持神经元发病机理之说。This paper presents 24 cases(19 males and 5 females)of myotonic dystrophy.Among them 21 cases were distributed in 5 pedigrees and 3 were sporadic cases.Age of the patients ranged between 20-28 but 2 were below 15.Clinical manifestations showed various symptoms,of multiple systems in addition to muscular atrophy and myotonia.Electromyography revealed myotonic potentiality.Muscular biopsy from 4 cases showed various degrees of degeneration and atrophy,but nerve endings and motor plates were normal.Thus,it does not support the theory of neurogenic pathogenesis.

关 键 词：强直性肌营养不良 肌萎缩 神经末梢 肌电图 系统病变 性连锁遗传病 肌肉萎缩 周围神经系统 肌肉生物电势图 电诊断图 家系调查 运动终板 神经-肌肉接头(点) 肌肉活检 肌强直 神经肌肉疾病 

分 类 号：R74[医药卫生—神经病学与精神病学]