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Congenital Bilateral Perisylvian syndrome
Background
The term congenital bilateral perisylvian syndrome (CBPS) describes a structural malformation of the brain. The underlying abnormality is known as ‘polymicrogyria’, which is a malformation of the cerebral cortex (outer layer of the brain). The term polymicrogyria designates an excessive number of small and prominent convolutions (folds) spaced out by shallow and enlarged sulci (grooves), giving the surface of the brain a lumpy aspect. Although it may be difficult to recognise mild forms of polymicrogyria on a magnetic resonance imaging (MRI) scan, infolding of the outer layer of the brain and secondary, irregular, thickening due to packing of microgyri (small folds) represent quite distinctive MRI characteristics.
Polymicrogyria may affect the whole or part of the cortex. Perisylvian polymicrogyria affects the Sylvian fissure, which contains the areas that control movements of the face, tongue and throat. It has become clear that this condition and the bulbar form of cerebral palsy or Worster-Drought syndrome are very closely related or part of a spectrum of the same condition. However, many children with Worster-Drought syndrome have normal MRI scans.
