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KMID : 0877219970010010119
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Journal of Korean Epilepsy Society
1997 Volume.1 No. 1 p.119 ~ p.124
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Schizencephaly : Clinical, Neuroradiologic and Electroencephalographic Features
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Song Hong-Ki
Koh Im-Seok
Park Se-Hyuck
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Abstract
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Purpose : Schizencephaly is an infrequent developmental disorder of cortical migration. Clinically it is often associated with epilepsy, developmental delay and motor weakness. We reviewed clinical, neuroimaging and EEG findings in the patients with schizencephaly to see the relations between those findings.
Subjects and Method : We investigated the type and location of the cleft, and associated brain anomalies as well as clinical manifestations in nine patients with schizencephaly. The presence of schizencephaly was established by means of magnetic resonance imaging (seven cases) and computed tomography (two cases). Serial EEGs during wakefulness and sleep state were recorded in seven patients.
Results : Three patients had bilateral clefts, another two patients had left unilateral open lip clefts, and the rest four patients had right open or closed lip clefts. Clinically, these patients presented epileptic seizure in 4 patients, motor dysfunction in 7 patients and variable developmental delay in 3 patients. Associated cerebral anomalies were seen in all patients except one. They included absence of septum pellucidum, gray matter heterotopia, polymicrogyria and hypoplasia of optic nerves. Interictal epileptiform discharges were observed in four patients with unilateral clefts. Two of four patients who had epileptic seizure showed focal spikes on EEG and focal spikes were observed in two crises without history of epileptic seizure. Focal spikes were present ipsilateral to the lesion slue in all cases.
Conclusion : In this study, the presence and severity of motor dysfunction is correlated with the type, size and location of the lesion, and the side of interictal spikes or unilateral slowing is correlated with the location of the lesion. Further case study should be necessary to support positive relationship between clinical or EEG findings and the nature of involved brain, in the patients with schizencephaly.
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KEYWORD
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Schizencephaly, Seizure, EEG, Neuronal migration
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