SCHIZENCEPHALY

Course overview

SCHIZENCEPHALY IS A RARE CONGENITAL NEURONAL MIGRATION DISORDER CHARACTERIZED BY A CLEFT LINED BY HETEROTOPIC GRAY MATTER, WHICH CONNECTS THE SURFACE OF THE CEREBRAL HEMISPHERE TO THE LATERAL VENTRICLE. SCHIZENCEPHALY WAS FIRST DESCRIBED BY WILMARTH IN 1887. THE TERM WAS COINED FROM THE GREEK WORD "SCHIZEN" 'TO DIVIDE' AND INTRODUCED BY YAKOVLEV AND WADSWORTH IN 1946, BASED ON THEIR WORK ON CADAVERS, THAT CLASSIFIED SCHIZENCEPHALY INTO TWO TYPES: CLOSED LIP AND OPEN LIP. IT MOST COMMONLY PRESENTS AS DEVELOPMENTAL DELAY, SEIZURES, AND WEAKNESS. THIS ACTIVITY REVIEWS THE ETIOLOGY, PATHOPHYSIOLOGY, EVALUATION, AND TREATMENT OF SCHIZENCEPHALY AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN THE CARE OF PATIENTS WITH THIS CONDITION.