Schizencephaly

What is Schizencephaly?

Schizencephaly is an extremely rare developmental birth defect characterised by abnormal slits, or clefts, in the cerebral hemispheres of the brain.  Babies with clefts in both hemispheres (called bilateral clefts) commonly have developmental delays, delays in speech and language skills, and problems with brain-spinal cord communication.  Individuals with clefts in only one hemisphere (called unilateral clefts) are often paralyzed on one side of the body, but may have average to near-average intelligence.  Individuals with schizencephaly may also have an abnormally small head, mental retardation, partial or complete paralysis, or poor muscle tone.  Most will experience seizures.  Some individuals may have an excessive accumulation of fluid in the brain called hydrocephalus. 

Is there any treatment?

Treatment generally consists of physical therapy and drugs to prevent seizures.  In cases that are complicated by hydrocephalus, a surgically implanted tube, called a shunt, is often used to divert fluid to another area of the body where it can be absorbed. 

What is the prognosis?

The prognosis for individuals with schizencephaly varies depending on the size of the clefts and the extent of neurological disabilities.