Aggressive confusional state as a clinical manifestation of status epilepticus in MELAS

Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS)1 is caused by mutations in the mitochondrial DNA (mtDNA), the most common at nucleotide 3243 in the tRNALeu(UUR).2 Although early development is typically normal, stroke-like episodes and seizures may occur in childhood or young adult life. Patients also may have short stature, exercise intolerance, hearing loss, dementia, pigmentary retinal degeneration, and migraine-like headache. A 43-year-old man had bilateral sensorineural hearing loss since adolescence and focal epilepsy since age 42 years. Before admission he had had transient left arm paresthesias and his wife had noted confusion and aggressive behavior. Neurologic examination revealed mild left arm paresis. The patient was aggressive and confused. Brain CT showed an inhomogeneous hypodensity in the right parietal region. MRI revealed cortical and subcortical hyperintensities in T2- and perfusion/diffusion-weighted images in the right parietal region; these findings were recent and compatible …