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Genetic and Rare Diseases Information Center (GARD)

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Greig cephalopolysyndactyly syndrome

Other Names for this Disease
  • GCPS
  • Greig syndrome
  • Polysyndactyly with peculiar skull shape
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Your Question

I am researching the link between Greig cephalopolysyndactyly syndrome, hydrocephalus, and seizures. I am curious about the reason for the hydrocephalus: is it a blockage issue or a slow draining issue?

Our Answer

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

What is the association of Greig cephalopolysyndactyly syndrome with hydrocephalus and seizures?

Seizures occur in less than 10% of individuals with Greig cephalopolysyndactyly syndrome.  Hydrocephalus is considered an uncommon feature of this condition.  However, when hydrocephalus is present, it increases the chances that seizures might develop.[1]
Last updated: 8/23/2011

What causes hydrocephalus in Greig cephalopolysyndactyly syndrome?

After an extensive search of the information resources available to us, we were unable to find any specific information on the cause of hydrocephalus in Greig cephalopolysyndactyly syndrome. However, individuals affected with this condition may have craniofacial features, which means the bones in the skull and face developed abnormally.[1]  Hydrocephalus occurs in up to 10% of individuals with craniofacial conditions. It is thought that changes in bony structures of the face and head may alter the drainage of fluid from the brain.[2]  In general, hydrocephalus is caused by the buildup of excess cerebrospinal fluid (CSF) in the brain due to a blockage in a passage through which the fluid normally flows.[3]  Visit the GARD page on hydrocephalus for more information and links to related resources.
Last updated: 8/23/2011