HYPOMELANOSIS OF ITO WITH BILATERAL PUTAM EN CALCIFICATION OR HEMIMEGALENCEPHALY

Two pediatric cases of hypomelanosis of Ito are reported. Associated a bnormalities were mental retardation, nonfebrile seizures, and hemimeg alencephaly detected by transfontanellar ultrasonography in one patien t and mental retardation, a quadripyramidal syndrome, and bilateral pu tamen calcification in the other. The cerebral abnormalities in these two patients are probably among the widely diverse neurologic manifest ations of hypomelanosis of Ito. The skin lesions characteristic of hyp omelanosis of Ito should be looked for routinely in children with cere bral malformations.