Migratory Rolandic Encephalopathy Caused by the Mitochondrial ND3 Variant

An 18-year-old woman was admitted to the hospital with focal seizures. Serial MRI studies showed migratory rolandic lesions (Figure, A–D) and the EEG showed sharp waves when the lesion appeared. The initial differential diagnosis included cerebral venous thrombosis, myelin oligodendrocyte glycoprotein antibody-associated disease, and metabolic disorders. Magnetic resonance venography, serum, and CSF analyses were unrevealing. An analysis of the entire mtDNA of urine sediment by next-generation sequencing showed a pathogenic m.10191T>C variant in the mt-ND3 gene (degree of heteroplasmy, 87%). Further analysis revealed the m.10191T>C variant was heteroplasmic in blood (24%) and oral mucosal (86%) cells. This case illustrates that m.10191T>C variant may present with migratory rolandic encephalopathy.1

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