Twelve epileptics with the age ranging from 4to 9 years who had typical absence without other epileptic seizures were treated with clonazepam for the periods from three to twenty-six months (average sixteen months) at the Department of Pediatrics, Keio University and the Second Tokyo National Hospital. In this series, ten fresh cases without history of previous therapy were treated with this drug only and in other two cases the seizures were intractable to conventional anticonvulsant drugs, to which clonazepam was newly added.
Age-dependent epilepsy in infancy is composed of infantile spasms and early-infantile epileptic encephalopathy with suppression-burst ( E. I. E. E. ). In this study, sleep states and changes in pulse, respiration and seizure activities during sleep states in infants with this type of epilepsy were examined electrophysiologically by means of overnight sleep polygraphy.
ACTH or hydrocortisone has been used in the treatment of the age-dependent epilepsy composed of infantile spasms and early-infantile epileptic encephalopathy with suppression-burst (E. I. E. E. ). In this study, the influence of ACTH or hydrocortisone therapy on sleep states, pulse, respiration and seizure activities of this epilepsy was examined electrophysiologically by means of overnight sleep polygraphy.
Clinical and pathological findings of a boy with Menkes kinky hair syndrome were reported. He showed progressive psychomotor disturbance with convulsion and peculiar hair abnormalities. X-rays of the bones showed metaphyseal spurring, flaring of the anterior end of the ribs and multiple Wormian bones. The levels of serum copper and ceruloplasmin were as low as one third of normal. He was in a vegetative state and died of pneumonia at the age of 20 months. Low copper content was found in the cerebrum and the liver at autopsy. But copper content of the red blood cell was normal and that of the duodenum was high.