Perisylvian syndrome

An X-linked dominant malformation of cortical development in which the brain surface is irregular and the normal gyral pattern replaced by multiple small, partly fused gyri separated by shallow sulci. Microscopic examination shows a simplified 4-layered or unlayered cortex. Neuologic findings include mild intellectual disability, epilepsy, and PSEUDOBULBAR PALSY. OMIM: 300388

Also Known As:

Networked: 3 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Hearing Loss (Hearing Impairment)
2.	Seizures (Absence Seizure)

Experts

1.	Agarwal, Aakanksha: 1 article (06/2017)
2.	Agarwal, Arun: 1 article (06/2017)
3.	Goyal, Manju: 1 article (06/2017)
4.	Jain, Jainendra: 1 article (06/2017)
5.	Axon, Patrick: 1 article (06/2007)
6.	Smith, Wendy: 1 article (06/2007)
7.	Bernardi, Bruno: 1 article (07/2006)
8.	Chugani, Harry T: 1 article (07/2006)
9.	Luat, Aimee F: 1 article (07/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Perisylvian syndrome:

1.	MagnesiumIBA 06/01/2017 - "In view of late onset postpartum seizures and non-responsiveness to magnesium sulphate, she was further evaluated and diagnosed to have congenital perisylvian syndrome(CPS). "
2.	Glucose (Dextrose)FDA LinkGeneric 07/01/2006 - "The clinical, magnetic resonance imaging, and 2-deoxy-2-[18F] fluoro-D-glucose (FDG) positron emission tomography scan findings of six children (age range: 3.2-16.7 years; 5 males) with congenital perisylvian syndrome were evaluated. " 07/01/2006 - "Congenital perisylvian syndrome: MRI and glucose PET correlations."

Therapies and Procedures

1.	Cochlear Implantation 06/01/2007 - "Cochlear implantation in a patient with Perisylvian syndrome."
2.	Cochlear Implants (Cochlear Implant) 06/01/2007 - "The method to determine whether the hearing loss is of cochlear or central origin and the progress of a child with Perisylvian syndrome who received a cochlear implant is discussed."