Status Epilepticus as the Initial Manifestation of Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke-Like Episodes Syndrome
- Author:
Joong-Goo KIM
1
;
Chul-Hoo KANG
;
Jay Chol CHOI
;
Jiyong SHIN
;
Min-Ju KIM
;
Jung-Hwan OH
Author Information
- Publication Type:증례
- From:Journal of the Korean Neurological Association 2020;38(4):276-280
- CountryRepublic of Korea
- Language:Korean
- Abstract: Mitochondrial encephalomyopathy with lactic acid and stroke-like episodes (MELAS) is a multisystem mitochondrial disorder that is rarely observed in adulthood. We report a case of MELAS syndrome diagnosed in a 22-year-old man presented with status epilepticus (SE) without a preceding stroke-like episode. Genetic testing revealed a mutation of heteroplasmic m.3243A>G. MELAS should be suspected in patients with recurrent, uncontrolled SE with unexplained severe lactic acidosis.
