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http://dx.doi.org/10.14253/acn.2022.24.2.84

Anterior canal-sparing bilateral vestibulopathy in MELAS syndrome

Kim, Jae-Myung (Department of Neurology, Chonnam National University Hospital, Chonnam National University Medical School)
Nam, Tai-Seung (Department of Neurology, Chonnam National University Hospital, Chonnam National University Medical School)
Lee, Seung-Han (Department of Neurology, Chonnam National University Hospital, Chonnam National University Medical School)

Publication Information

Annals of Clinical Neurophysiology / v.24, no.2, 2022 , pp. 84-89 More about this Journal

Abstract

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.

Keywords

Bilateral vestibulopathy; MELAS syndrome; Head impulse test;

Citations & Related Records

Reference

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