MULTICYSTIC ENCEPHALOPATHY - REVIEW OF 8 CASES WITH ETIOLOGIC CONSIDERATIONS

Multicystic encephalomalacia (MCE) is a rare lesion that arises during the perinatal period. Although hypoxic-ischemic insults may be respon sible for this lesion, recent evidence suggests that herpesviruses may represent another etiologic agent. To elucidate the pathogenesis of M CE, eight cases collected over a 34-year period were evaluated for des tructive lesions in gray and white matter. Immunocytochemical methods, in situ hybridization and polymerase chain reaction (PCR) methodology were employed to search for herpes simplex viruses types 1 and 2 (HSV 1 and HSV2), cytomegalovirus (CMV), varicella tester virus (VZV), Epst ein-Barr virus (EBV) and JC variant of papovavirus (JCV). Review of th e clinical histories revealed that there had been a complicated labor and delivery in 6/7 cases. Neuropathological lesions consisted of exte nsive tissue destruction, neuronal loss and gliosis in hemispheric whi te matter, cerebral cortex, basal ganglia, thalamus, cerebellum and br ainstem tegmentum. Only one case showed evidence of latent HSV infecti on by PCR. CMV, VZV JCV and EBV were not detected. Arteriopathy was no ted in one case. The widespread nature of the lesions and their associ ation with perinatal ischemia suggest that severe hypoxia may be the m ore common etiology of MCE. Term infants appear especially susceptible to this type of cerebral damage.