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Congenital Bilateral Perisylvian Syndrome

Abstract

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NORD is very grateful to Phillip L. Pearl, MD, Division Chief, Child Neurology, Children's National Medical Center; Professor of Pediatrics and Neurology, The George Washington University School of Medicine, for assistance in the preparation of this report.

Synonyms of Congenital Bilateral Perisylvian Syndrome

  • CBPS

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Congenital bilateral perisylvian syndrome (CBPS) is an extremely rare neurological disorder that may be apparent at birth (congenital), infancy, or later during childhood. It is characterized by partial paralysis of muscles on both sides (diplegia) of the face, tongue, jaws, and throat (pseudobulbar palsy); difficulties in speaking (dysarthria), chewing (mastication), and swallowing (dysphagia); and/or sudden episodes of uncontrolled electrical activity in the brain (epilepsy). In most cases, mild to severe intellectual disability is also present. Associated symptoms and findings are thought to be due to improper development of the outer surface of the brain (cerebral cortex) during embryonic growth (neuronal dysmigration). In most cases, the disorder appears to occur randomly for unknown reasons (sporadically).

Organizations related to Congenital Bilateral Perisylvian Syndrome

[Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder (e.g., epilepsy or mental retardation).]

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