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Schizencephaly Information Page

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What is Schizencephaly?
Is there any treatment?
What is the prognosis?

What is Schizencephaly?
Schizencephaly is an tremendously rare developmental disorder characterized by abnormal slits, or clefts, in the brain's cerebral hemispheres. Schizencephaly is a form of porencephaly in which there is a cyst or cavity in the cerebral hemispheres. Individuals with clefts in both hemispheres (bilateral clefts) are commonly developmentally delayed and have delayed speech and language skills and corticospinal dysfunction. Individuals with smaller, unilateral clefts (clefts in only one hemisphere) are often paralyzed on one side of the body and may have normal intelligence. Patients with schizencephaly may also have varying degrees of microcephaly (abnormally small head), mental retardation, hemiparesis or quadriparesis (partial or complete paralysis), and reduced muscle tone (hypotonicity). Most patients have seizures. Some may have hydrocephalus.

Is there any treatment?
Treatment for individuals with schizencephaly usually consists of physical therapy, treatment for seizures, and, in cases that are complicated by hydrocephalus, a shunt (a surgically implanted tube that diverts fluid from one pathway to another).

What is the prognosis?
The prognosis for individuals with schizencephaly varies depending on the size of the clefts and the degree of neurological deficit.

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