J Korean Neurol Assoc.  1991 Sep;9(3):367-373.

A Case of Motor Neuron Disease with Hypogonadotropic Hypogonadism-A Variatn of Kennedy-Alter-Sung Syndrome?

• Park KD
• Sunwoo IN
• Chi JG

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.

MeSH Terms

Adult
Feminization
Follow-Up Studies
Humans
Hypogonadism
Korea
Lower Extremity
Male
Motor Neuron Disease*
Motor Neurons*
Muscles