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J Korean Neurol Assoc. 2004;22(5):520-523.
A Case of Pure Word Deafness in MELAS Syndrome
Dong-Sun Shin,
Department of Neurology, Chung-Ang University College of Medicine, Seoul, Korea
순수어농을 보인 MELAS 증후군 1예
신동선, 권오상 이현옥 윤영철
중앙대학교 의과대학 신경과학교실
Abstract
An 18-year-woman was referred with seizure activity and global aphasia. Diagnosis of MELAS syndrome with left temporo-parieto-occipital infarction was confirmed by gene analysis. Global aphasia was improved completely. Right temporal infarction developed after 5 months. Diagnosis of pure word deafness was made in view of impaired verbal comprehension in presence of adequate reading, writing, spontaneous speaking, and well preserved environmental sound perception. MRI findings of the patient support the hypothesis of the pathway of non-verbal hearing in previous reports. Key Words: Pure word deafness, Cortical deafness, MELAS syndrome

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