Schizencephaly is a rare congenital neurological condition characterized by the presence of abnormal clefts in one or both cerebral hemispheres of the brain. The clefts may be open or close lipped, and extend from the surface of the brain down to the ventricles. Bilateral clefts are the more severe form of the condition, and affected individuals present with delays in physical and speech development. Patients with unilateral clefts are less severely affected, and predominantly show paralysis of one side of the body. Other features seen in affected patients include microcephaly, mental retardation, hypotonia, spasticity, seizures, and hydrocephalus.
Imaging studies like CAT and MRI are used to diagnose the condition. There is no cure for schizencephaly; treatment being supportive. Seizures can be controlled with anti-convulsant medications, but may in some cases require surgical removal of some amount of brain tissue. The hydrocephalus can be managed by a surgical shunt that can remove excess fluid from the brain. Prognosis varies for affected individuals. Severely affected patients may require long-term care, and generally have a short life-span.