Fact-sheet: Schizencephaly
Updated on 04/21/2016 at 11:20 PM
View all RADEOS cases associated with this fact-sheet
Definition
Malformation of the central nervous system related to the presence of a unilateral or bilateral cleft extending from the lateral ventricle to the cortical surface, lined by dysplastic gray matter.
May be related to various conditions (infections, especially CMV, vascular causes, maternal trauma, toxic exposure, etc.).
Schizencephaly may be:
- unilateral or bilateral: bilateral in more than 50% of cases.
- open-lip: communicating the subarachnoid spaces with the lateral ventricles.
- closed-lip: no communication, "incomplete cleft"
Preferential locations:
- frontal
- parietal
Clinical features
Unilateral cleft: - hemiparesis - mild motor deficit - epilepsy Bilateral cleft: - severe developmental delay - paresis - spasticity - epilepsy
CT
Visualization of a unilateral or bilateral cleft extending from the lateral ventricle to the cortex
Search for calcifications (CMV context)
MRI
SE T1: - characterize closed-lip vs open-lip cleft - demonstration of dysplastic gray matter lining the cleft. SE T2: - allows better differentiation between ectopic gray matter foci (T2 hypointense) lining the cleft and delayed myelination. T2*: Search for associated calcifications (CMV context). SE T1 with gadolinium: Search for associated developmental venous anomalies. MRA: The middle cerebral artery may "drop" into the cleft.
Management
Treatment of epilepsy.
Support for psychomotor development.
Classification
Unilateral/bilateral
Open-lip/closed-lip