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Multicystic encephalopathy: review of eight cases with etiologic considerations
K M Weidenheim1, S R Bodhireddy, G J Nuovo
1Department of Pathology (Neuropathology), Albert Einstein College of Medicine, Bronx, New York 10461.
Abstract:
Multicystic encephalomalacia (MCE) is a rare lesion that arises during the perinatal period. Although hypoxic-ischemic insults may be responsible for this lesion, recent evidence suggests that herpesviruses may represent another etiologic agent. To elucidate the pathogenesis of MCE, eight cases collected over a 34-year period were evaluated for destructive 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 (HSV1 and HSV2), cytomegalovirus (CMV), varicella zoster virus (VZV), Epstein-Barr virus (EBV) and JC variant of papovavirus (JCV). Review of the clinical histories revealed that there had been a complicated labor and delivery in 6/7 cases. Neuropathological lesions consisted of extensive tissue destruction, neuronal loss and gliosis in hemispheric white matter, cerebral cortex, basal ganglia, thalamus, cerebellum and brainstem tegmentum. Only one case showed evidence of latent HSV infection by PCR. CMV, VZV, JCV and EBV were not detected. Arteriopathy was noted in one case. The widespread nature of the lesions and their association with perinatal ischemia suggest that severe hypoxia may be the more common etiology of MCE. Term infants appear especially susceptible to this type of cerebral damage.
Insights
Multicystic encephalomalacia (MCE) is a rare perinatal brain lesion. Severe hypoxia is the likely cause, though herpesvirus was investigated, with term infants being most susceptible.
Area of Science:
- Neuropathology
- Perinatal Medicine
- Virology
Background:
- Multicystic encephalomalacia (MCE) is a rare perinatal brain lesion.
- While hypoxic-ischemic insults are suspected, herpesvirus has been proposed as an alternative etiology.
- Understanding the pathogenesis of MCE is crucial for identifying at-risk infants.
Purpose of the Study:
- To investigate the etiology and pathogenesis of MCE.
- To evaluate neuropathological findings in MCE cases.
- To detect specific herpesviruses and other viral agents in affected brain tissue.
Main Methods:
- Analysis of eight MCE cases over 34 years.
- Immunocytochemistry, in situ hybridization, and PCR for viral detection (HSV1, HSV2, CMV, VZV, EBV, JCV).
- Review of clinical histories and neuropathological examination of brain tissue.
Main Results:
- Extensive neuropathological lesions observed, including tissue destruction, neuronal loss, and gliosis in multiple brain regions.
- Only one case showed evidence of latent herpes simplex virus (HSV) infection via PCR.
- Cytomegalovirus (CMV), varicella zoster virus (VZV), JC variant of papovavirus (JCV), and Epstein-Barr virus (EBV) were not detected.
- Complicated labor and delivery noted in most cases (6/7).
- Arteriopathy present in one case.
Conclusions:
- Severe perinatal hypoxia is suggested as the more common etiology of MCE.
- Herpesvirus infection is unlikely to be a primary cause in most MCE cases.
- Term infants are particularly vulnerable to this type of cerebral damage.