Annals of Clinical Neurophysiology

The Korean Society of Clinical Neurophysiology (대한임상신경생리학회)
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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)
  • Received : 2022.07.18
  • Accepted : 2022.08.11
  • Published : 2022.10.30
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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

Acknowledgement

This study was performed in accordance with the recommendations of the Institutional Review Board of the Chonnam National University Hospital (Gwangju, South Korea).

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