Dyke-Davidoff-Masson syndrome in adulthood

A 54-year-old woman was seen due to weakness and gait difficulty. Physical examination revealed spasticity (Ashworth 2–3), weakness (3/5), hyperactive deep tendon reflexes on the right side, and facial asymmetry. MRI of the brain was consistent with Dyke-Davidoff-Masson Syndrome (DDMS) (figure). DDMS ensues due to an insult to the developing brain, characterized by cerebral hemiatrophy, contralateral hemiparesis, unilateral thickening of the skull, widening paranasal sinuses, fascial asymmetry, and mental retardation. Diagnosis of DDMS is very uncommon in adulthood.1,2 This unusual presentation suggests that a late diagnosis of DDMS may occur.