A Case of Adynamia Episodica Hereditaria

J Korean Neurol Assoc > Volume 1(1); 1983 > Article

Journal of the Korean Neurological Association 1983;1(1): 101-105.

가족력 동반한 고칼륨혈증 주기성마비 1예

허균, 최일생, 김기환

연세대학교 신경과.

A Case of Adynamia Episodica Hereditaria

Kyoon Hu, M.D., Il-Saeng Choi, M.D., Ki-Hwan Kim, M.D.

Department of Neurology, Yeonsei University

Abstract

Four members of a family suffering from frequent attacks of flaccid paralysis with asymptomatic myotonia were evaluated. There was an autosomal dominant pattern of inheritance and their earliest symptoms were noticed between 1-3 years of age. The plasma potassium level rised during the attack and an EMG demonstrated myotonic discharge. Treatment with acetazolamide was beneficial. These findings were consistent with adynamia episodica hereditaria. The clinical features and pathogenesis were also described.

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