Epidermal nevus syndrome with isolated enlargement of one temporal lobe: a case report

A 5-month-old girl presented with infantile spasms and psychomotor retardation. She was born with epidermal nevi on the right side of the head, the right arm and the back. MRI of the head showed a right hemimegalencephaly limited to the temporal lobe. There were no MRI signs of neocortical dysplasia. Ultrasonography revealed multiple cysts in both ovaria and a small solid lesion in the left adrenal gland. Hemimegalencephaly represents an overgrowth phenomenon; the isolated temporal lobe hypertrophy can be explained as a mild expression of hemimegalencephaly. To our knowledge this has not been described before.