ストークス・アダムス発作を呈し，経静脈的ペースメーカ植込みにて改善した思春期筋緊張性ジストロフィー症の一例

An adolescent case of myotonic dystrophy who developed Stokes-Adams attacks successfully treated with transvenous DDD pacemaker

　筋緊張性ジストロフィー症は遺伝性神経筋疾患で，筋強直と筋萎縮を主症状とするが，その他にも，白内障，内分泌障害，中枢神経障害，心障害を合併することが知られている。

　心障害ことに刺激伝導系障害は本疾患における生命的予後に直接かかわるため注意すべき合併症であるが，通常30〜40歳代になり始めて発現することが多く，小児期や思春期における心合併症の報告はほとんどない。

Myotonic dystrophy is a progressive autosomal dominant disorder characterized by myotonia and degeneration of skeletal muscle. Cardiac involve-ment is also noted.

In general, the systemic disease, which begins in second or third decades of life, is present for several years before cardiac manifestations become overt.

A fifteen-year-old girl developed syncopal attacks frequently and her His bundle electrogram revealed prolonged HV interval. Transient complete heart block was suspected to the cause of Stokes-Adams attacks. Transvenous DDD pacemaker was success-fully implanted and symptoms related to arrhythmia disappeared.