Schizencephaly: correlation of clinical findings with MR characteristics

To correlate clinical outcome with the size and location of clefts in patients with schizencephaly.

MR scans and clinical records of 20 patients with schizencephaly were retrospectively reviewed. Seven patients had bilateral clefts (10 open lip clefts, 4 closed lip clefts), eight patients had right-sided unilateral clefts (5 open lip clefts, 3 closed lip clefts), and five patients had left-sided unilateral clefts (3 open lip clefts, 2 closed lip clefts).

Statistically significant correlations were found as follows: Patients with bilateral schizencephalies had significantly worse intellectual (P = .004) and speech (P = .03) development than those with unilateral clefts; patients with unilateral large or medium open lip schizencephalies had significantly worse motor (P = .003) and intellectual (P = .008) impairment than those with unilateral closed lip or small open-lip schizencephalies; patients with frontal lobe involvement had a significantly higher incidence of motor dysfunction than those without frontal lobe involvement (P = .01). Strong similarities were noted in the patient outcomes and the locations of cortical anomalies of patients with schizencephaly and those with nonschizencephaly focal cortical dysplasias.

A common pathogenetic origin for the formation of focal cortical dysplasia in the form of polymicrogyria and schizencephalies is proposed. Patients with small unilateral schizencephalies have a good developmental prognosis, particularly when the motor cortex is not involved.