[A case of Dyke-Davidoff-Masson syndrome with total hemiatrophy]

We reported a 39-year-old man with Dyke-Davidoff-Masson syndrome presenting with total hemiatrophy. The patient had a muscle defect in the left occipital lesion at birth and left hemiatrophy including the face in his infancy. The present illness consists of atrophy of the left face, trunk and extremities, a muscle defect accompanied with scleroderma of the left dorsal cervical lesion, bilateral pes cavus, cleft palate, left hemiparesis, including facial palsy and diminished superficial sensation of the left side of the body. Decrease in right cranial volume and slight elevation of the right temporal pyramid and the superior border of the orbit were seen on the X-ray study of the head. Atrophy of the right cerebrum and cerebral peduncle was seen on magnetic resonance imaging. These findings suggest that hemiatrophy of the brain was responsible for total hemiatrophy of the body in this patient. The etiology of cerebral hemiatrophy is not known. There is only a report of Dyke-Davidoff-Masson syndrome accompanied with total hemiatrophy.