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裂脳症4例の臨床像と神経放射線学的所見を比較・検討した。症例1は右側がopen-lip,左側がclosed-lipの両側性裂脳症,症例2は対側に異所性灰白質のある左傍中心溝部のclosed-lipの片側性裂脳症,症例3は左傍中心溝部のclosed-lipの片側性裂脳症,症例4はclosed-lipの両側性裂脳症で,症例3以外は両側性に病変がみられた。全例知能レベルに異常はなく,症例1と3で軽度の片麻痺があるのみで,日常生活に支障はなかった。全例てんかんを伴っていたが,抗けいれん剤でコントロールは良好であった。MRIで裂脳症の所見が両側にあっても,臨床的に軽症な症例が存在する。
We report the clinical and neuroimaging features of 4 cases with schizencephaly. Case 1 had bilateral schizencephaly with open-lip on the right and closed-lip on th left. Case 2 had unilateral schizencephaly with closed-lip on the left and subcortical heterotopia on the right. Case 3 had unilateral schizencephaly with closed lip on the left. Case 4 had bilateral closed-lip schizencephaly. Although all cases except for Case 3 had bilateral lesions, neurodevelopmental outcome was generally good; Case 1 and 3 had mild hemipare-sis. All patient have epilepsy which are well-controlled with antiepileptic drugs.
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