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Congenital varicella-zoster. A serologically proven case with necrotizing encephalitis and malformation
1Department of Neuropathology, Hospital for Sick Children, London, Great Britain.
Insights
Congenital varicella-zoster virus infection following maternal chickenpox can cause severe neurological damage and developmental abnormalities in newborns. This case highlights the critical impact of prenatal viral exposure on fetal development.
Area of Science:
- Neurology
- Infectious Diseases
- Developmental Pediatrics
Background:
- Maternal chickenpox during pregnancy poses risks to fetal development.
- Varicella-zoster virus (VZV) can cross the placenta, leading to congenital infection.
- Early pregnancy VZV exposure is associated with congenital varicella syndrome.
Observation:
- A male infant born after maternal chickenpox at 14 weeks gestation presented with low birth weight, hypotonia, and limb contractures.
- Clinical signs included muscle wasting, areflexia, and evidence of widespread denervation on electromyogram.
- Congenital VZV infection was confirmed by rising varicella-zoster-specific IgM titres.
Findings:
- Neuropathological examination revealed extensive destructive and inflammatory lesions in the central nervous system, including the cerebral cortex, white matter, thalamus, brain stem, and spinal cord.
- Cerebellar heterotopias and bilateral polymicrogyric malformations of the insular cortex were noted.
- The infant succumbed to the infection at 8 days of age.
Implications:
- This case underscores the severe neurodevelopmental consequences of congenital varicella-zoster virus infection.
- Prenatal diagnosis and management strategies for maternal VZV infection are crucial.
- Further research into preventative measures and therapeutic interventions for congenital VZV is warranted.
Abstract:
Following maternal chicken pox in the 14th week of pregnancy, a male infant was born with low birth weight, muscle wasting and limb contractures, hypotonia and areflexia. A rising titre of varicella-zoster-specific IgM (by enzyme-linked immunoabsorbent assay) confirmed congenital infection, and electromyogram showed widespread denervation. Death occurred at 8 days. Neuropathological examination revealed destructive and inflammatory lesions of cerebral cortex and white matter, thalamus, brain stem and spinal cord. In addition there were cerebellar heterotopias and bilateral polymicrogryic malformations of the insular cortex.