저성선자극호르몬성 성선기능저하증에 동반된 운동신경원질환 1예 보고; Kennedy-Alter-Sung 증후군의 변형?

Abstract

This is the first report of motor neuron disease associated with feminization in Korea. The case in this report is a 26 year old male patient wlth hypogonadotropic hypogonadism who developed progressive lower motor neuron dysfunctions started from the lower extremities and later spreaded to the entire body including bulbar muscles. At first glance, wve thought that this is very similar to well known Kennedy-Alter-Sung syndrome but after thorough evaluation and follow up of the partient for 4 years until his death we concluded that this case is somewhat different from Kennedy-Alter-Sung syndrome in several aspects even though it is similar and may be a variant of that syndrome or a new unique syndrome associated with hypogonadotropic hypogonadism.