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.

Intracranial hypotension is a benign disorder characterized by orthostatic headache. It is caused by CSF leakage, therefore its treatment of choice has been epidural blood patch when initial conservative treatments were not effective. We would like to report a 26-year-old patient with recurrent orthostatic intracranial hypotension in spite of the successful epidural blood patch several times. Her headache was caused by myelography 8 month ago. Cisternography showed a CSF leakage at the level of L1 vertebral body. Headache was resolved completely after epidural blood patch and then recurred 3 times several months after blood patch. Now, she is free from headache for 5 months after last blood patch.