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Developmental bilateral perisylvian dysplasia.
M I Shevell1, L Carmant, K Meagher-Villemure
1Division of Pediatric Neurology, Montreal Children's Hospital, Quebec, Canada.
Pediatric Neurology
|July 1, 1992
Summary
Foix-Chavany-Marie syndrome, a rare neurological disorder, presents in acquired and developmental forms. This study details a newborn with developmental bilateral perisylvian dysplasia, characterized by polymicrogyria, contributing to understanding this rare condition.
Area of Science:
- Neuroscience
- Developmental Neuroscience
- Neuropathology
Background:
- Foix-Chavany-Marie syndrome (FCMS) is typically acquired, affecting the anterior operculum and causing expressive dysphasia and pseudobulbar palsy.
- A congenital variant of FCMS, linked to neuronal migration disorders, has been recently described.
- Understanding the pathogenesis of FCMS is crucial for diagnosis and potential therapeutic strategies.
Observation:
- This report presents a case of a newborn diagnosed with developmental bilateral perisylvian dysplasia.
- Autopsy and detailed histologic examination confirmed polymicrogyria, aligning with the rare pathological findings of this syndrome.
- The study highlights the clinical heterogeneity, noting both neonatal and childhood presentations of this disorder.
Findings:
- The newborn case confirms developmental bilateral perisylvian dysplasia as a pathological correlate of the congenital variant of FCMS.
- Histologic evidence of polymicrogyria supports a restricted disorder of neuronal migration as the underlying cause.
- Clinical presentation varies, with cases observed from neonatal period through childhood.
Implications:
- This case reinforces the concept of a developmental, congenital form of FCMS stemming from neuronal migration abnormalities.
- Pathogenesis may involve genetic factors disrupting neuronal migration or in utero vascular events.
- Further research into the genetic and environmental factors is warranted to elucidate the etiology of FCMS.