Investigators at University of Toronto and McMaster University, Canada, report a 22-year-old woman with multiple episodes of status epilepticus and migratory cortical stroke-like lesions. Ketogenic diet and magnesium resulted in seizure freedom and decrease in frequency of stroke-like episodes following improvement of mitochondrial dysfunction. Initial mitochondrial genetic testing was negative. Diagnosis was established by muscle biopsy for mitochondrial genome sequencing, demonstrating a mitochondrial DNA disease-causing mutation. [1]

COMMENTARY. A ketogenic diet should be considered for treatment of intractable seizures and stroke-like episodes related to mitochondrial respiratory chain complex (MRC) defects. In a Korean study of 14 patients with MRC defects and various seizure types (5 with infantile spasms, 4 with Lennox-Gastaut syndrome, 1 with Landau-Kleffner syndrome), 50 – 90% seizure control was obtained with the ketogenic diet [2]. A subsequent study in Korea involving 24 cases of MRC defect with seizures, the ketogenic diet controlled seizures in 75% patients [3].