儿童神经肌肉疾病与其复健精.pptVIP

兒童神經肌肉疾病及其復健 天主教台東聖母醫院 物理治療師 陳志軒 今日主題 何謂神經肌肉疾病? 基本解剖學介紹 臨床上常見的兒童神經肌肉病變 復健的處理 中樞神經系統 人體的運動單位 人體的運動單位 前角細胞(ant. horn cell ) 脊髓神經根(spinal roots) 週邊神經(peripheral nerve) 神經肌肉交界處(neuromuscular junction) 肌肉(muscle) 神經肌肉病變 泛指運動單位(motor unit)的某一部分因病變的影響, 而造成臨床上 肌肉無力 張力過低 基腱反射降低 肌肉萎縮 等下運動神經元症候群的症狀 兒童常見的神經肌肉病變 肌肉疾病 裘馨氏肌肉萎縮症 貝克型肌肉萎縮症 運動神經元疾病 小兒麻痺 脊髓性肌肉萎縮症 脊髓膨出症 週邊神經 臂神經叢受損 裘馨氏肌肉萎縮症 Duchenne muscular dystrophy (DMD) Duchenne muscular dystrophy (DMD) is caused by an altered gene found on the X-chromosome, so the condition usually affects only boys. In some cases, the disorder appears out of the blue, following a new mutation in the unfertilised egg. In others, DMD is inherited from a carrier mother, who is usually unaffected herself. A carrier mother has a one-in-four chance of having an affected son and a one-in-four chance of having a carrier daughter. 學齡前可發現的現象 Gower’s sign 骨盆及軀幹肌肉無力的現象 常出現在DMD的小孩 肌肉組織切片 脊椎變形 貝克型肌肉萎縮症 Becker Muscular Dystrophy Symptoms: ??Generalized weakness and muscle wasting affecting limb and trunk muscles first. Calves often enlarged. Almost identical to Duchenne but often much less severe. Can be significant heart involvements. Onset: Adolescence or adulthood Progression: Disease progresses slowly and with variability but will affect all voluntary muscles. Survival well into mid to late adulthood Calf muscles initially enlarge (an attempt by the body to compensate for loss of muscle strength), but the enlarged muscle tissue is eventually replaced by fat and connective tissue (pseudohypertrophy). Cardiomyopathy may occur, but the development of congestive heart failure or arrhythmias (irregular heartbeats) is rare. X-linked recessive (females are carriers Becker Muscular Dystrophy (BMD) is one of the nine types of muscular dystrophy and is considered the more mild cousin of Duchenne Muscular Dystrophy, the most common inherited childhood lethal disorder of humankind. BMD disease affects approxi