A Case of MELAS Syndrome Involving Diabetes Mellitus, Cerebellar Ataxia and Progressive Sensorineural Hearing Loss

Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a maternally inherited mitochondrial disorder. MELAS manifests different clinical symptoms due to heteroplasmy individual susceptibility tends be aggravated by metabolic events. It presents various manifestations including hearing impairment, diabetes mellitus, stroke like episodes. However, date, only few cases of accompanied with cerebellar ataxia have been reported in Korea. We hereby present an unusual case patient diagnosed involving sensorineural loss, which were stresses such as pregnancy hypoglycemic shock.