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KMID : 1044320220240020084
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Annals of Clinical Neurophysiology
2022 Volume.24 No. 2 p.84 ~ p.89
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Anterior canal-sparing bilateral vestibulopathy in MELAS syndrome
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Kim Jae-Myung
Lee Seung-Han
Nam Tai-Seung
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Abstract
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Vestibular dysfunction has rarely been reported in MELAS syndrome. A 40-year-old male with long-term diabetes and hearing loss experienced a stroke-like episode with hemisensory disturbance and lactic acidosis. Brain MRI showed temporo-parieto-occipital cortical lesions, and a final diagnosis was made of MELAS syndrome with the mitochondrial 3243A>G mutation. Neuro-otologic evaluations revealed anterior-canal-sparing bilateral impairments of the vestibulo-ocular reflex in the video head impulse test and no caloric paresis. This unique pattern of vestibular dysfunction may aid in diagnosing MELAS syndrome.
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KEYWORD
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Bilateral vestibulopathy, MELAS syndrome, Head impulse test
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