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Diseases

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

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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 1.54 per 100,000 individuals.[1] This information was published in 2005 in a report of a population-based study of schizencephaly involving 4 million births in the state of California from 1985 to 2001.[2]
Last updated: 5/24/2011

What are the effects of seizures on development?

Both clinical and laboratory studies demonstrate that seizures early in life can result in permanent behavioral abnormalities.[3] In general, 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 higher mortality rates than the normal population, but their lower survival rates are most often due to the underlying condition, not the epilepsy itself. The studies on the effects of seizures on memory and learning vary widely and depend on many factors. 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, and emotional and behavioral disorders, occur in a significant number of children with epilepsy.[4] 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.[5]
Last updated: 1/17/2011

What is the prognosis for individuals with schizencephaly?

The prognosis for individuals with schizencephaly varies depending on the size and location of the clefts and the extent of neurological disabilities.[6] Severe seizures are quite common, as is spasticity. Children with bilateral clefts (clefts in both hemispheres) typically have severe mental and psychomotor developmental delay; wide clefts usually correlate with moderate to severe developmental delay; and children with narrow or closed-lipped lesions may only have hemiplegia and/or seizures, with no developmental delay.[7]
Last updated: 5/24/2011

References