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MELAS Syndrome (A Case Report)
MELAS증후군 (증례보고)

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Authors
Myung, Ho Jin; Kim, Jong Sung; Seo, Yeon Lim; Chi, Je G.
Issue Date
1988-09
Publisher
Seoul National University College of Medicine
Citation
Seoul J Med 1988;29(3):315-322
Keywords
MELAS syndromeKorean case
Abstract
MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis,
and stroke-like episode) is a rare and currently incompletely defined mitochondrial disease
involving mainly muscle and brain. We have recently seen a 17-year-old male patient who,
we believe, is the first Korean case. The patient showed the classical picture of MELAS: short
stature, generalized limb weakness, lactic acidemia, basal ganglia calcification, migraine-like
headache and recurrent strokes with subsequent hemiparesis, hemianopia, and seizures.
Electronmicroscopic examination confirmed the diagnosis by demonstrating numerous abnormal
muscle mitochondria which contained paracrystalline inclusion bodies. A brief discussion
of current status, inherent problems, and possible pathogenic mechanisms of this syndrome will conclude our report. ; MELAS증후군은 최근 기술된 사립체질환의 하나로서 사립체성 근육 및 뇌질환, 유산증, 뇌졸중양 증상 등을 특징으로 한. 저자들은 최근 우리나라의 첫 증례로 생각되는 환자를 경험하였기에 보고하는 바이다. 환자는 왜소한 체구의 17세 남자로서 근무력, 유산증, 기저핵의 석회화, 편두통양 두통, 수 회의 뇌졸증 및 이에 따른 반신마비, 시야결손, 경련 등의 증상을 나타내었다. 근육생검상 경한 근육질환이 나타났으며, 전자현미경 소견상 봉입체를 갖는 비정상적인 사립체가 관찰되었다.
ISSN
0583-6802
Language
English
URI
http://hdl.handle.net/10371/6285
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 29 No.3 (1988)
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