Two infants, six and seven months of age, with West syndrome associated with cerebral tumors are reported from the Department of Neurology, Pediatric Hospital, Buenos Aires, Argentina. Initial neurologic examinations were normal and the diagnosis of the tumors was by ultrasound and CT. One infant had a Grade III glioma in the right thalamus and the other had an anaplastic ependymoma and cyst in the right hemisphere. EEGs revealed generalized hypsarrhythmia in both cases. Infantile spasms responded to ACTH 5 IU/kg/day. One patient died at 18 months of age and the other was seizure free after complete surgical resection of the ependymoma. [1]
COMMENT. Other brain tumors associated with West syndrome have included choroid plexus papilloma, ganglioglioma, and optic nerve glioma. Infantile spasms and choroid plexus papilloma have also been described in Aicardi syndrome (see Ped Neurol Briefs October 1989; 3:74).