Bilateral frontoparietal polymicrogyria
Other Names for this Disease
- Cerebellar ataxia with neuronal migration defect
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Your QuestionWhat is bilateral frontoparietal polymicrogyria? What are its symptoms? How is it different from bilateral perisylvian polymicrogyria in how it presents and what symptoms we see?
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Bilateral frontoparietal polymicrogyria (BFPP) is a rare neurological disorder that affects the cerebral cortex (the outer surface of the brain). BFPP specifically affects the frontal and parietal lobes on both sides of the brain (bilateral). Signs and symptoms typically include moderate to severe intellectual disability, developmental delay, seizures, cerebellar ataxia, strabismus, and dysconjugate gaze (eyes that are not aligned). Some cases are caused by mutations in the GPR56 gene and are inherited in an autosomal recessive manner. Treatment is based on the signs and symptoms present in each person.
Last updated: 6/8/2015
The signs and symptoms of bilateral frontoparietal polymicrogyria vary but may include:
Last updated: 6/7/2015
The signs and symptoms of bilateral perisylvian polymicrogyria (BPP) vary but may include:
Last updated: 6/5/2015
- Chang B, Walsh CA, Apse K & Bodell A. Polymicrogyria Overview. Gene Reviews. August 6, 2007; http://www.ncbi.nlm.nih.gov/books/NBK1329/. Accessed 3/9/2015.
- POLYMICROGYRIA, BILATERAL FRONTOPARIETAL; BFPP. OMIM. April 2014; http://omim.org/entry/606854.
- Congenital Bilateral Perisylvian Syndrome. NORD. 2015; http://rarediseases.org/rare-diseases/congenital-bilateral-perisylvian-syndrome/.
- Kilinc O, Ekinci G, Demirkol E3, Agan K. Bilateral agenesis of arcuate fasciculus demonstrated by fiber tractography in congenital bilateral perisylvian syndrome. Brain Dev. March 2015; 37(3):352-355.