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[Cytomegalovirus fetal infection. Porencephaly with polymicrogyria in a 15-year-old boy]
I Tominaga1, M Kaïhou, T Kimura
1Service de Neuropsychiatrie, Hôpital National de Chiba, Japon.
Insights
This study details a case of congenital cytomegalovirus (CMV) infection in a male infant, leading to severe neurological complications including polymicrogyria and developmental delay. The findings suggest neuronal migration failure as a potential cause of polymicrogyria.
Area of Science:
- Neurology
- Virology
- Developmental Pediatrics
Background:
- Congenital cytomegalovirus (CMV) infection is a significant cause of non-genetic sensorineural hearing loss and neurodevelopmental disabilities.
- Maternal rubella history in a prior pregnancy highlights potential risks of viral teratogens during gestation.
Observation:
- A neonate presented with splenomegaly, microcephaly, chorioretinitis, and signs of immaturity.
- Imaging revealed a left temporal lobe cyst and periventricular calcifications; seizures and spastic rigidity were noted by 8 months.
- Postmortem examination confirmed polymicrogyria, a left temporal lobe cavity, heterotopias, and periventricular white matter calcifications.
Findings:
- Laboratory and serological tests confirmed fetal cytomegalovirus infection.
- Neuropathological findings included polymicrogyria, heterotopias, and calcifications, but lacked typical viral inclusions.
- Absence of gliosis in polymicrogyric cortex suggests neuronal migration failure rather than post-migrational injury.
Implications:
- This case underscores the severe neurological sequelae of congenital CMV infection.
- The findings support the hypothesis that polymicrogyria may result from impaired neuronal migration during fetal development.
- Further research into the mechanisms of neuronal migration failure in congenital infections is warranted.
Abstract:
A boy, born after 41 weeks of gestation, presented with splenomegaly, microcephaly and chorioretinitis accompanied by immaturity signs. His mother was in good health but her previous pregnancy had been aborted owing to rubella. Laboratory data, including serological and virological evidence, confirmed the diagnosis of fetal cytomegalovirus infection. CT scan indicated a large cyst in the left temporal lobe and periventricular calcifications. At about 8 months of age, convulsions were noticed which were not controlled effectively by medication. There was spastic rigidity without significant psychomotor development. He died at the age of 15. Postmortem neuropathological examination revealed polymicrogyria predominant in the right cerebral hemisphere as well as a large cavity in the left temporal lobe communicating with the lateral ventricle. Widespread heterotopias and calcifications were observed notably in the periventricular white matter. No typical inclusion was found. By the method of Holzer and GFAP immunocytochemistry, no gliosis was noted in the cerebral cortex having the feature of polymicrogyria. This might support the theory that polymicrogyria is caused by neuronal migration failure.