原因不明の心疾患を伴ったKugelberg-Welander病の一例

Kugelberg-Welander Disease with Cardiopathy of Unknown Etiology. Clinical Report of a Case

はじめに

　Kugelberg-Welander病^1）（以下K-W病と記す）は，1952年，初めて独立疾患として記載され，以来我が国でも，現在まですでに60数例の報告がみられる。^2）しかしこの疾患が心症状を伴うという報告はほとんどみられない。最近，われわれは原因不明の心拡大を呈し，著明なうっ血性心不全を伴ったK-W病の1臨床例を経験したので報告する。

A case of Kugelberg-Welander dizease (K-W) with cardiomegaly and congestive heart failure is reported. Patient is a 23-year-old male who de-veloped unsteady gait at the age of 3 and diagnosis of progressive muscular dystrophy was made at 6. But progression of gait disturbance was gradual until the age of 23, when the operation for elonga-tion of achilles tendon was performed because of contracture, and thereby he was noted abdominal dis-tension and cardiomegaly. Following operation, he was referred to our clinic for further evaluation of muscle atrophy and cardiac disease.