Hostname: page-component-76fb5796d-wq484 Total loading time: 0 Render date: 2024-04-26T08:29:11.029Z Has data issue: false hasContentIssue false
  • English
  • Français

Lower motor neuron involvement in perisylvian polymicrogyria

Published online by Cambridge University Press:  18 September 2006

Maria Clark ,
Matthew Pitt  and
Brian GR Neville
Maria Clark
Affiliation:
Neurosciences Unit, University College London Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, London, UK.
Matthew Pitt
Affiliation:
Neurosciences Unit, University College London Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, London, UK.
Brian GR Neville
Affiliation:
Neurosciences Unit, University College London Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, London, UK.
Get access

Abstract

Congenital bilateral perisylvian polymicrogyria syndrome (CBPS) has a cerebral cortical localization and its phenotype was thought to be purely central. This study of seven children with CBPS (five males, two females; mean age 5y [SD 3y 6mo]; range 1mo–11y 10mo) documents electrophysiological evidence of lower motor neuron involvement in association with congenital contractures (limb or jaw) in six of the seven children studied. This is not an expected association and does not conform to the traditional lesional classification system of the cerebral palsies. Possible pathogenic mechanisms are discussed but this association of upper and lower motor neuron involvement is likely to be a previously unsuspected part of a genetic or other pathogenic sequence.

Type
Original Articles
Information
Developmental Medicine and Child Neurology , Volume 48 , Issue 10 , October 2006 , pp. 842 - 846
Copyright
2006 Mac Keith Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)