schizencephaly
Pronunciation: skitz-en-SEF-ah-lee
A rare brain malformation characterized by abnormal clefts or splits in the cerebral cortex that extend from the brain surface to the ventricles.
Full Definition
Schizencephaly is a congenital brain malformation consisting of gray matter-lined clefts that extend through the full thickness of the cerebral hemisphere, from the pial surface to the ventricular system. These clefts can be unilateral or bilateral and are classified as either closed-lip (where the cleft walls touch) or open-lip (where the cleft walls are separated by cerebrospinal fluid). The condition is thought to result from abnormal neuronal migration or vascular insults during early brain development. Clinical manifestations vary widely depending on the size, location, and laterality of the clefts, ranging from mild hemiparesis to severe developmental delays and intractable seizures.
Usage
Usage note: Not to be confused with schizophrenia; the prefix refers to splitting or clefting of brain tissue.
In Context
- "The MRI demonstrated unilateral open-lip schizencephaly in the left hemisphere." — Pediatric radiology report
- "Bilateral schizencephaly was associated with severe developmental delay and refractory seizures." — Clinical case summary