A Case of Kennedy Syndrome Mimicking Myasthenia Gravis

J Korean Neurol Assoc > Volume 19(5); 2001 > Article

Journal of the Korean Neurological Association 2001;19(5): 544-546.

중증근무력증과 유사한 구척수 근위축증(Kennedy 증후군)1예

이병주, "권기한.김성민나해리이병철류상효배재천김지인*이이진성 †"

한림대학교 의과대학 신경과학교실,국립보건원 유전질환과*,연세의대 소아과학교실

A Case of Kennedy Syndrome Mimicking Myasthenia Gravis

"Byung-Ju Lee, M.D., Ki-Han Kwon, M.D., Sung-Min Kim, M.D., Ha-ry Na, M.D., Byung-Chul Lee, M.D., Sang-Hyo Ryu,M.D., Jae-Chun Bae, M.D., Jee-In Kim.*, Jin-Sung Lee, M.D. †"

"Department of Neurology, Hallym University College of Medicine Division of Genetic Disease, National Institute of Health, Korea* Department of Pediatrics, Yonsei University College of Medicine †"

Abstract

"Patients with Kennedy syndrome, which progresses more slowly than amyotrophic lateral sclerosis show a mild degree of motor fluctuation but rarely show significant decremental responses to repetitive nerve stimulations. Even in a patient with decremental responses to repetitive nerve stimulations, there is usually no significant improvements in motor symptoms to anticholinesterases. We experienced a patient with Kennedy syndrome, who showed significant decremental responses to repetitive nerve stimulations and a marked degree of motor fluctuation. His motor fluctuation responded dramatically to anticholinesterase. J Korean Neurol Assoc 19(5):544~546, 2001 Key Words : Kennedy syndrome, Decremental response, Anticholinesterase"