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Genetic and Rare Diseases Information Center (GARD)

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Schizencephaly


Other Names for this Disease

  • Familial schizencephaly
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Your Question

How many cases of schizencephaly have been reported in Florida, and worldwide? Will my child always have develomental delay, and will this get worse with every seizure? What is the prognosis for this condition?

Our Answer

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

How many cases of schizencephaly have been reported worldwide, and in the state of Florida?

The number of cases of schizencephaly that have been reported in Florida or worldwide is not currently known. However, the estimated prevalence of schizencephaly is 1/64,935 births in the USA and 1/69,444 in UK.[1]
Last updated: 11/18/2014

What are the effects of seizures on development?

The long-term effects of seizures vary widely depending on the seizure's cause. Children whose epilepsy is a result of a specific condition (such as schizencephaly) have lower survival rates than the normal population, but this is most often due to the underlying condition.[2]

Both clinical and laboratory studies show that seizures early in life can result in permanent behavioral abnormalities.[3] In general, the earlier a child has seizures and the more extensive the area of the brain affected, the poorer the outcome. Children with seizures that are not well-controlled are at higher risk for intellectual decline. Learning and language problems as well as emotional and behavioral disorders occur in a significant number of children with epilepsy.[2] It is worth noting, however, that progressive mental deterioration is often related to the neurologic disorder that caused the seizures rather than to the seizures themselves.[4]
Last updated: 11/18/2014

What is the long-term outlook for people with schizencephaly?

The long-term outlook for people with schizencephaly varies depending on the size and location of the clefts and the extent of intellectual disabilities.[5][1] For example, children with a small cleft in one hemisphere may have paralysis on one side of the body and little to no intellectual disability, while clefts in both hemispheres can lead to quadriplegia (paralysis of both arms and legs) and severe intellectual disability.[1]
Last updated: 11/18/2014

References
Other Names for this Disease
  • Familial schizencephaly
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.