NEONATAL DIAGNOSIS OF TUBEROUS SCLEROSIS

Intracranial tumors are rare in the neonatal period, and generally the most common histological types are astrocytoma, medulloblastoma, chor oid plexus papilloma and neuroectodermal tumors. The early diagnosis o f these tumors is often very difficult. The authors report a case of a full-term newborn who presented with opisthotonus. A subependymal mas s was detected by cerebral ultrasonography, and when the child was 1 m onth of age depigmentations appeared on the trunk and on the right leg , confirming the suspicion of tuberous sclerosis. At 3 months of age t he child suffered infantile spasm with hypsarrhythmia. The development al delay, the marked progressive neurological deterioration and the da ily seizures suggested surgical resection. Histologic studies showed a subependymal giant cell astrocytoma such as typically occurs in tuber ous sclerosis.