进行性神经性肌萎缩又称为排骨肌萎缩症或Charcot-Marie-Tooth氏病,是一种家族遗传性疾病。Charcot和Marie于1886年首先将本病从许多进行性肌萎缩疾病中分离出来,作为一独立疾病进行讨论。同年Tooth也以“腓骨肌型进行性肌萎缩”为题对本病加以论述。1889年Hoffman提出将本病取名为“进行性神经性肌萎缩”。本病较为罕见,国内至今共有18例报道。我院今年收治本病患者4例。本文除报告我院4例外,并 Progressive neuromuscular atrophy, also known as chondromalacia dystrophy or Charcot-Marie-Tooth’s disease, is a familial hereditary disease. Charcot and Marie first isolated the disease from a number of progressive amyotrophic diseases in 1886 for discussion as an independent disease. The same year, Tooth also “fibular muscle progressive muscle atrophy,” the title of the disease to be discussed. Hoffman 1889 proposed the disease was named “progressive neuromuscular atrophy.” The disease is relatively rare, so far a total of 18 cases reported. Our hospital admitted to this disease in 4 cases this year. In addition to this report in our hospital 4 cases, and