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KMID : 0361020230660060400
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Korean Journal of Otolaryngology - Head and Neck Surgery
2023 Volume.66 No. 6 p.400 ~ p.403
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A Case of MELAS Syndrome Involving Diabetes Mellitus, Cerebellar Ataxia and Progressive Sensorineural Hearing Loss
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Kang Sun-Seong
Lee Woong-Woo
Choi Jung-Ho
Shim Hyun-Joon
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Abstract
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Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndromeis a maternally inherited mitochondrial disorder. MELAS syndrome manifests different clini-cal symptoms due to mitochondrial heteroplasmy and individual susceptibility and tends to beaggravated by metabolic events. It presents various clinical manifestations including hearingimpairment, diabetes mellitus, and stroke like episodes. However, to date, only a few cases ofMELAS syndrome accompanied with cerebellar ataxia have been reported in Korea. Wehereby present an unusual case of a patient diagnosed with MELAS syndrome involving dia-betes mellitus, cerebellar ataxia and sensorineural hearing loss, which were aggravated bymetabolic stresses such as pregnancy and hypoglycemic shock.
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KEYWORD
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Cerebellar ataxia, Diabetes mellitus, MELAS syndrome, Mitochondria, Sensorineural hearing loss
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