A case of Menkes kinky hair disease presenting with pseudoperiodic tonic spasms during the clinical course is described. The reported case had been treated by parenteral copper administration since 10 days of age. Although he gradually appeared to deteriorated mentally and developmentally and developed West syndrome at eight months of age, he had no convulsive seizures until four years of age. At four years of age, tonic spasms, which tended to be i.e. pseudoperiodic appeared. He had two types of tonic spasms, flexor spasms and extensor spasms. An ictal EEG also revealed different patterns, including diffuse desynchronization superimposed over the low voltage fast activities of during the flexor spasms and fast activities appeared only during extensor spasms. Both of these ictal events were dominantly in the left hemisphere on a EEG and each of the tonic spasms was easily controlled with phenytoin. The possibility must be entertained that the presented tonic spasms may have been partial seizures
