Endocrinol Metab > Volume 23(6); 2008 > Article
Journal of Korean Endocrine Society 2008;23(6):444-449.
DOI: https://doi.org/10.3803/jkes.2008.23.6.444    Published online December 1, 2008.
A Case of MELAS Syndrome Manifested by Insulin-deficient Diabetes Mellitus.
Hee Seog Jeong, Ji Young Kang, Hyun Kim, Kyu Ho Lee, Dal Sic Lee, Guan Yong Choi, Tae Geun Oh, Hyen Jeong Jeon
Department of Internal Medicine, Chungbuk National University College of Medicine, Korea.
Abstract
MELAS (mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes) syndrome is characterized by stroke-like episodes before the age of 40, encephalopathy, seizures, dementia and lactic acidosis, and is caused by mutations in mitochondrial DNA. Diabetes mellitus and cardiac involvement are also frequently seen in MELAS syndrome. It is a classic mitochondrial disorder that shows a slow, chronic, progressive course, and presents with multiple organ involvement including the central nervous system, skeletal muscle, eye, cardiac muscle and gastrointestinal system.
Key Words: diabetes mellitus, MELAS syndrome, mitochondrial disease


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