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Genetic and Rare Diseases Information Center (GARD)

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Bilateral perisylvian polymicrogyria


Other Names for this Disease
  • CBPS
  • Congenital bilateral perisylvian syndrome
  • Perisylvian syndrome
  • Perisylvian syndrome, congenital bilateral
  • PMGX
Related Diseases
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Your Question

What 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?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is bilateral frontoparietal polymicrogyria?

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.[1][2]
Last updated: 6/8/2015

What are the signs and symptoms of bilateral frontoparietal polymicrogyria?

The signs and symptoms of bilateral frontoparietal polymicrogyria vary but may include:[2][1]
  • Moderate to severe intellectual disability
  • Developmental delay
  • Seizures
  • Dysconjugate gaze (eyes that are not aligned)
  • Ataxia
  • Strabismus
  • Increased muscle tone
  • Finger dysmetria (difficulty controlling speed, distance and/or power of movements)
Last updated: 6/7/2015

What are the signs and symptoms of bilateral perisylvian polymicrogyria?

The signs and symptoms of bilateral perisylvian polymicrogyria (BPP) vary but may include:[3][4]
  • Partial paralysis of muscles on both sides of the face, tongue, jaws, and throat 
  • Dysarthria
  • Difficulty chewing
  • Dysphagia
  • Mild to severe intellectual disability
  • Seizures and/or epilepsy
  • Sudden, involuntary spasms of facial muscles
  • Developmental delay
Last updated: 6/5/2015

References
Other Names for this Disease
  • CBPS
  • Congenital bilateral perisylvian syndrome
  • Perisylvian syndrome
  • Perisylvian syndrome, congenital bilateral
  • PMGX
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.