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Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe
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Lower motor neuron involvement in perisylvian polymicrogyria

Maria Clark1, Matthew Pitt, Brian G R Neville

  • 1Neurosciences Unit, University College London Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, London, UK.

Developmental Medicine and Child Neurology
|September 19, 2006
PubMed

Insights

Congenital bilateral perisylvian polymicrogyria syndrome (CBPS) typically affects the brain

Area of Science:

  • Neurology
  • Genetics
  • Developmental Biology

Background:

  • Congenital bilateral perisylvian polymicrogyria syndrome (CBPS) is a brain malformation.
  • Its phenotype was traditionally considered exclusively central nervous system-related.
  • This limited the understanding of its full clinical spectrum.

Purpose of the Study:

  • To investigate potential non-central nervous system manifestations in CBPS.
  • To explore the association between CBPS and motor neuron involvement.
  • To challenge the traditional classification of cerebral palsies.

Main Methods:

  • Electrophysiological studies were conducted on seven children with CBPS.
  • Clinical assessments focused on neurological signs and congenital contractures.
  • Patient data included age, sex, and clinical history.

Main Results:

  • Six of seven children with CBPS showed electrophysiological evidence of lower motor neuron involvement.
  • This involvement was associated with congenital contractures of the limbs or jaw.
  • The findings suggest a broader phenotype than previously recognized.

Conclusions:

  • CBPS may involve both upper and lower motor neuron pathways.
  • This dual involvement is not typically expected in CBPS.
  • The findings suggest a potential genetic or pathogenic sequence previously unrecognized in CBPS.

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