Bilateral perisylvian polymicrogyria
Other Names for this Disease
- Congenital bilateral perisylvian syndrome
- Perisylvian syndrome
- Perisylvian syndrome, congenital bilateral
Bilateral perisylvian polymicrogyria (BPP) is a rare neurological disorder that may be apparent at birth, infancy, or later during childhood. It is characterized mainly by partial paralysis of muscles on both sides of the face, tongue, jaws, and throat; difficulties in speaking, chewing, and swallowing (dysphagia); and/or seizures (epilepsy). In most cases, mild to severe intellectual disability is also present. While the exact cause of BPP is not fully understood, this condition is thought to be due to improper development of the outer surface of the brain (cerebral cortex) during embryonic growth. In most cases, BPP appears to occur sporadically (non-inherited) for unknown reasons in a person with no family history of the disorder. However, there are reports of a few families in which more than one member has BPP. Environmental causes of the disorder can include exposure to cytomegalovirus (CMV) during pregnancy. Treatment usually focuses on the specific symptoms that are present in each individual and may include anticonvulsant drugs to help prevent, reduce, or control epilepsy associated with the condition.
Last updated: 1/15/2014
- Perisylvian Syndrome, Congenital Bilateral. NORD. December 31, 2010; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Perisylvian%20Syndrome%2C%20Congenital%20Bilateral. Accessed 5/6/2011.
- Genetics Home Reference (GHR) contains information on Bilateral perisylvian polymicrogyria. This website is maintained by the National Library of Medicine.
- The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
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