Perisylvian syndrome

Common Name(s)

Perisylvian syndrome, Bilateral perisylvian polymicrogyria

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.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Perisylvian syndrome" for support, advocacy or research.

PMG Awareness Organization, Inc.

The PMG Awareness Organization is an international non-profit organization of families, caregivers, and medical professionals who care about those with Polymicrogyria. Our mission is to promote awareness, diagnosis, and the treatment of Polymicrogyria through education, research, advocacy, and support for individuals with Polymicrogyria, their families, and other concerned parties.

Last Updated: 20 Feb 2013

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Perisylvian syndrome" for support, advocacy or research.

PMG Awareness Organization, Inc.

The PMG Awareness Organization is an international non-profit organization of families, caregivers, and medical professionals who care about those with Polymicrogyria. Our mission is to promote awareness, diagnosis, and the treatment of Polymicrogyria through education, research, advocacy, and support for individuals with Polymicrogyria, their families, and other concerned parties.

http://www.pmgawareness.org

Last Updated: 20 Feb 2013

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General Resources

PMG Awareness Educational Pamphlet Inside

Educational resource about Polymicrogyria

Updated 16 Dec 2012

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PMG Awareness Education Pamphlet Outside

Educational Pamphlet about polymicrogyria

Updated 16 Dec 2012

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PMG Awareness' FaceBook GROUP [age

This is our FaceBook GROUP support page that allows family, friends, and supporters to post and receive feedback from other members of the group.

Updated 13 Jan 2013

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Blogs

PMG Awareness Organization's Blog

PMG Awareness Organizational Blog

Updated 16 Dec 2012

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Perisylvian syndrome" returned 14 free, full-text research articles on human participants. First 3 results:

A case of Bilateral Perisylvian Syndrome with reading disability.
 

Author(s): Mark A Eckert, Virginia W Berninger, Fumiko Hoeft, Kenneth I Vaden,

Journal: Cortex. 2016 Mar;76():121-4.

 

Last Updated: 31 Dec 1969

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Anterior corpus callosotomy combined with anterior temporal resection with amygdalohippocampectomy: outcome in a patient with congenital bilateral perisylvian syndrome.
 

Author(s): Zhu Junming, Zhao Yuanyuan, Feng Fang, Fu Weiming, Hays Ryan, Zhang Jianmin, Feng Li, Jin Xiao, Chen Shuda

Journal: Turk Neurosurg. 2014 ;24(1):70-4.

 

Congenital bilateral perisylvian syndrome (CBPS) is characterized by epilepsy, cognitive deficits, pseudobulbar palsy and diplegia of the facial, pharyngeal and masticatory muscles. Epilepsy has been described in nearly 90% of affected patients. The epilepsy is usually severe and ...

Last Updated: 31 Dec 1969

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Bilateral perisylvian ulegyria: an under-recognized, surgically remediable epileptic syndrome.
 

Author(s): Lucas P Schilling, Renata R Kieling, Tharick A Pascoal, Hyoung-Ihl Kim, Min Cheol Lee, Yun Hee Kim, Eliseu Paglioli, Pedro R Neto, Jaderson C Costa, Andre Palmini

Journal: Epilepsia. 2013 Aug;54(8):1360-7.

 

Interest in the association of epilepsy and pseudobulbar palsy was rekindled since the identification through magnetic resonance imaging (MRI) of bilateral perisylvian polymicrogyria (PMG). Seizures are often intractable, but resective epilepsy surgery has not been recommended. However, ...

Last Updated: 31 Dec 1969

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Perisylvian syndrome" returned 0 free, full-text review articles on human participants.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

There are currently no open clinical trials for this condition.