All issues > Volume 33(11); 1990

Original Article

J Korean Pediatr Soc. 1990;33(11):1586-1592. Published online November 30, 1990.

A Case of MELAS Syndrome.

Ki Joong Kim¹, Yong Seung Hwang¹, Young In Choi², Sung Hye Park³, Je Geun Chi³

¹Department of Pediatrics, Seoul National University, College of Medicine Seoul, Korea
²Department of Family Medicine, Seoul National University, College of Medicine Seoul, Korea
³Department of Pathology, Seoul National University, College of Medicine Seoul, Korea

Received: May 17, 1990;  Accepted: July 24, 1990.

Abstract

MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode) syndrome is a rare but clinicopathologically distinctive mitochondrial disorder. We experienced a case of MELAS syndrnmp in A 11 year-old girl who showed repeated attacks of stroke-like episodes, vomitng, cortical blindness, hearing deficit and alternating hemiplegia. Serum lactic acid level was elevated (6.4 mmol/Z). The diagnosis was confirmed by muscle biopsy which revealed dispersed ragged-red fibers.

Keywords :Mitochondrial myopathy, MELAS syndrome