Two Cases of Familial Hypokalemic Periodic Paralysis

J Korean Neurol Assoc > Volume 8(1); 1990 > Article

Journal of the Korean Neurological Association 1990;8(1): 180-184.

저칼륨성 가족성 주기성마비 2예

김광국, 이재홍,이상수,노재규,이상복,명호진

서울대학교 신경과.

Two Cases of Familial Hypokalemic Periodic Paralysis

Kwang Kuk Kim, M.D., Jae Hong Lee, M.D., Sang Soo Lee, M.D., Jae Kyu Roh, M.D., Sang Bok Lee, M.D., Ho Jin Myung, M.D.,

Department of Neurology, College of Medicine,Seoul National University

Abstract

Two patients of a family, suffenng from frequent periodic paralysis, were evaluated. Their family history reveals seven affected members in three generations, with autosomal dominance pattern. Their earliest symptoms were noticed between 10-13 years of age. The serum potassium level fell during the attack. EMG demonstrated progresslve reduction in numer of motor units and drop off in the response of muscle fibers to stimuli. These findings were consistent with familial hypokalemic periodic paralysis. The clinical features, electron microscoscopic findings, and pathogenesis were also described.