Clinical, imaging features and follow-up study of schizencephaly in 35 children

Abstract

Objective To summarize the correlation between clinical manifestations and imaging characteristics of schizencephaly in order to provide a basis for the diagnosis and prognosis. Methods Thirty-five outpatients with schizencephaly diagnosed at Department of Pediatric Neurology of Henan Provincial People′s Hospital from January 2009 to May 2015 were retrospectively selected, and they were divided into different groups (patients with unilateral lesions or bilateral lesions) according to their cranial magnetic resonance imaging (MRI) features.The clinical manifestations were compared between different groups. Results Of 35 patients, 19 (54.3%) patients were male and 16 (45.7%) patients were female.Twenty-four patients were found with unilateral lesions(68.6%), including 16 cases (66.7%) with hemiparesis and 8 cases(33.3%) with no motor impairment.Mental retardation was observed in 8 patients (33.3%) and 6 patients (25.0%) showed speech impairment.Eleven patients were found the bilateral lesions (31.4%), including 5 patients (45.4%) with tetraparesis, 4 patients (36.4%) with hemiparesis, and 2 patients (18.2%) with no motor impairment.Mental retardation was observed in 9 patients (81.8%) and 9 patients (81.8%) showed speech impairment.There were significant differences in motor impairment, mental retardation and speech impairment between the unilateral lesion group and bilateral lesion group (Z=-2.40, P=0.002; χ2=7.09, P=0.012; χ2=9.94; P=0.003). Epileptic seizure occurred in 18 patients (51.4%). Binary Logistic regression analysis indicated that cortical dysplasia beyond the cleft and open-lip lesions were the major risk factors for seizures (OR=4.44, 2.73; P=0.005, 0.029). Imaging characteristics: there were closed-lip lesions in 10 patients (28.6%), open-lip lesions in 21 patients (60.0%) and open/closed-lip lesions in 4 patients (11.4%). Anatomic localization of all clefts was found in the frontal lobes in 19 patients (54.3%), in frontoparietal lobes in 11 patients (31.4%), in pa-rietal lobes in 2 patients (5.7%), and in occipital lobes in 3 patients (8.6%). After 1-6-year follow-up, 12 patients treated with antiepileptic drug were seizure - free (all with unilateral lesions), and 6 patients had refractory epilepsy (3 patients with bilateral lesions). Conclusions Schizencephaly is a rare structural disorder of cerebral cortical development.Those with bilateral lesions are usually manifested with severe motor, speech impairment and mental retardation and their prognosis is poor.Schizencephaly patients complicated with cortical dysplasia beyond the cleft or open-lip lesions are more easily attacked by seizures.MRI plays an important role in the diagnosis and prognosis judgment of schizencephaly. Key words: Schizencephaly; Epilepsy; Hemiplegia; Prognosis