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Bilateral opercular polymicrogyria.

P S Becker1, A M Dixon, J C Troncoso

  • 1Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD.

Annals of Neurology
|January 1, 1989
PubMed
Summary
This summary is machine-generated.

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Foix-Chavany-Marie syndrome (FCMS) is a rare disorder affecting facial and throat muscles. This study presents the first pathological findings of the developmental form, revealing specific brain abnormalities.

Area of Science:

  • Neurology
  • Developmental Neuroscience
  • Pathology

Background:

  • Foix-Chavany-Marie syndrome (FCMS), also known as faciopharyngoglossomasticatory diplegia, is an uncommon neurological disorder.
  • It typically arises from bilateral lesions in the anterior opercula, which can be vascular or developmental in origin.

Observation:

  • This study provides the first pathological documentation of the developmental variant of FCMS.
  • Examination focused on the brain structures affected in this rare developmental condition.

Findings:

  • Pathological examination revealed bilateral failure of opercular closure, a key developmental defect.
  • Additional findings included opercular polymicrogyria, periventricular gray-matter heterotopias, and absence of the septum pellucidum.

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Implications:

  • These findings advance the understanding of the neuropathological basis of developmental FCMS.
  • This detailed pathological description can aid in diagnosing and potentially managing this rare syndrome.
  • It highlights the importance of opercular development in neurological function.