THE CUTIS VERTICIS GYRATA AND MENTAL RETARDATION SYNDROME IN A 4-YEAR-OLD BOY

A 4-year-old profoundly mentally retarded boy with cutis verticis gyrata is described. He had four folds on the left parietal region, small asymmetric head, micrognathia, short neck, marmorated skin, thoracic scoliosis, spastic tetraplegia and epilepsy. Encephalography revealed marked asymmetric macroventriculy with hypoplastic brain stem and cerebellum. Thorn's test and corticotrophin stimulation test gave normal responses. The boy is the youngest patient with the cutis verticis gyrata and mental retardation syndrome described in the literature. The numerous malformations of the patient strongly support the theory of prenatal origin of the cutis verticis gyrata and mental retardation syndrome.