Kuntzer T, Lettry-Trouillat R, Bogousslavsky J
Service de neurologie, Centre Hospitalier Universitaire Vaudois, Lausanne.
Rev Med Suisse Romande. 2000 Sep;120(9):725-31.
The percentage and cause of neuromuscular (NM) diseases have been analysed during a 15-year period of time. A NM disorder was found in 10,852 patients (or 14.4% of all neurological patients seen in our hospital). Mononeuropathies have been the most common causes, followed by polyneuropathies and radiculopathies, but with a variable percentage during time for the most frequent causes, that were carpal tunnel syndrome and diabetic polyneuropathies. Myopathies, diseases of the neuromuscular junction and anterior horn cell disorders counted for 5% among all NM disorders during the first 10 years (1% of all neurological patients) and for 12% during the last 5 years (2.4% of all neurological patients), this period corresponds to the opening of our outpatient clinic dedicated to NM diseases. Among polyneuropathies, the most common causes were diabetes mellitus, chronic inflammatory demyelinating polyneuropathies and the inherited forms of peripheral neuropathies. Among myopathies, the most frequently reported symptom was exercise-induced myalgia. The 2 most common muscular dystrophies (MD) were Steinert myotonic MD, and facio-scapulo-humeral MD. Other myopathies were rare, but of diverse causes, all of them corresponding to the newly introduced definition of Rare or Orphan Diseases.
我们对15年间神经肌肉疾病的发病率及病因进行了分析。在10852例患者中发现了神经肌肉疾病（占我院所有神经科患者的14.4%）。单神经病是最常见的病因，其次是多发性神经病和神经根病，但最常见病因（腕管综合征和糖尿病性多发性神经病）的发病率随时间变化。在最初10年里，肌病、神经肌肉接头疾病和前角细胞疾病占所有神经肌肉疾病的5%（占所有神经科患者的1%），而在最后5年里占12%（占所有神经科患者的2.4%），这一时期恰逢我院开设了神经肌肉疾病门诊。在多发性神经病中，最常见的病因是糖尿病、慢性炎症性脱髓鞘性多发性神经病和遗传性周围神经病。在肌病中，最常报告的症状是运动诱发的肌痛。最常见的两种肌肉萎缩症是斯坦纳特强直性肌营养不良症和面肩肱型肌营养不良症。其他肌病较为罕见，但病因多样，均符合罕见病或孤儿病的新定义。
