A Schizencephaly abstract written in 1997
Schizencephaly
Correlations of clinical and radiologic features
- A. Maurine Packard, MD,
- Van S. Miller, PhD, MD and
- Mauricio R. Delgado, MD, FRCPC
-
doi: 10.1212/WNL.48.5.1427 Neurology May 1997 vol. 48 no. 5 1427-1434
- Abstract
- Full Text (PDF)
Abstract
Schizencephaly is an uncommon congenital
disorder of cerebral cortical development. Although a well-recognized
cause of seizures
and developmental deficits in children, previous
reports describe the range of neurode-velopmental outcome in only 47
patients.
We report the clinical and cranial imaging features
of 47 children with unilateral open-lip (171, unilateral closed-lip
(121,
bilateral open-lip (121, and bilateral closed-lip
(6) schizencephaly, as defined radiologically. The schizencephalic cleft
occurred more often in the anterior than in the
posterior neocortex. Children with closed-lip schizencephaly presented
with
hemiparesis or motor delay whereas patients with
open-lip schizencephaly presented with hydrocephalus or seizures.
Forty-three
patients (91%) had associated cerebral
developmental anomalies, most commonly absence of the septum pellucidum
(45%) and focal
cortical dysplasia (40%). There was a history of
seizures in 57% of cases, a third of which were classified as difficult
to
control. Neurodevelopmental outcome was generally
poor, with 51% of patients (24/47) having severe deficits, 32% of
patients
(15/47) having moderate impairment, and 17% of
patients (8/47) having mild or no problems. Patients with closed-lip
schizencephaly
were more likely to have a mild to moderate outcome
than those with open-lip type (78% versus 31%; p < 0.05). Children with unilateral schizencephaly had a mild or moderate outcome more frequently than those with bilateral
lesions (62% versus 28%; p < 0.05).
Children who had involvement of a single lobe accounted for 88% of those
with mild outcomes and 53% of those with
moderate outcomes. Unilateral closed-lip
schizencephaly was associated with the best neurodevelopmental outcome;
in contrast,
11 of 12 children with bilateral open-lip clefts
had severe disabilities. Language development was significantly more
likely
to be normal in those children with unilateral
schizencephaly than in those with bilateral clefts (48% versus 6%; p
< 0.002). Thus, the presentation and outcome of children with
schizencephaly are quite variable but are related to the extent
of cortex involved in the schizencephalic defect.
- © 1997 by the American Academy of Neurology
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