Abstract
A Korean female, delivered at full term by forceps, weighed 2, 650g. During pregnancy the mother hadhad a severe headache in the first trimester, but otherwise no particular symptoms. After birth the infant never cried, and moderate rigidity of the extxtremities, trismus, ocular fixa tion and absence of pupillary light reflex were noted. The papillae of the optic nerve were pale, but head circumference and plain skull films were normal. The patient developed fever, petechiae on the trunk and hepatosplenomegaly and expi red suddenly during PEG at 3 months of age. Laboratory studies revealed pleocytosis and markedly elevated protein (500-1, 400mg/dl) in the CSF, increased IgM fraction and dislocation of both hips. Just before death the PEG films showed remarkable periventricular calcificationsof the lateral ventricles, which were greatly dila ted, and thinning of the cortex.
Autopsy findings showed marked thinning of the cortex and severe meningoencephalitis with moderate calcification, interstitial pneumonia, hepatitis, pmn cell infiltration of the spleen etc.
Serologically and histopathologically, toxo plasmosis, cytomegalovirus and fungal infections were ruled out. An infectious agent was not identified but some virus, thought to be the cause of prenatal systemic generalized infection of the fetus was suspected to be the causative organism.
