本文選題：腦裂畸形 + 癲癇��； 參考：《鄭州大學(xué)》2017年碩士論文

【摘要】：目的總結(jié)腦裂畸形患兒的臨床表現(xiàn)與影像學(xué)特征之間的聯(lián)系,并進行隨訪了解預(yù)后差異,為本病診斷及預(yù)后判斷提供參考依據(jù)。方法分析2009年1月至2015年5月在河南省人民醫(yī)院小兒神經(jīng)內(nèi)科門診就診的35例腦裂畸形患兒資料,并進行隨訪,根據(jù)頭顱磁共振(MRI)特征進行分組(單、雙側(cè)腦裂畸形),比較臨床特征。結(jié)果35例腦裂畸形患兒中,男19例(54.3%),女16例(45.7%);單側(cè)腦裂畸形24例(68.6%),其中偏癱16例(66.7%),無運動障礙8例(33.3%);認(rèn)知發(fā)育缺陷8例(33.3%),語言障礙6例(25.0%)。雙側(cè)腦裂畸形11例(31.4%),其中四肢癱5例(45.4%),偏癱4例(36.4%),無運動障礙2例(18.2%);認(rèn)知發(fā)育缺陷9例(81.8%),語言障礙9例(81.8%)。比較運動發(fā)育缺陷程度、認(rèn)知發(fā)育缺陷及語言障礙的有無在單、雙側(cè)腦裂畸形的差異有統(tǒng)計學(xué)意義(P=0.002,P=0.012,P=0.003)。癲癇發(fā)作18例(51.4%),二元Logistic回歸分析示:伴裂隙以外部位皮質(zhì)發(fā)育不良(P=0.005,OR=4.44,CI:1.57-12.57)、開唇型腦裂畸形(P=0.029,OR=2.73,CI:1.11-6.74)是癲癇發(fā)作的主要危險因素。影像學(xué)特征:閉唇型10例(28.6%),開唇性21例(60.0%),混合型4例(11.4%);裂隙位于額部19例(54.3%),額頂部11例(31.4%),頂部2例(5.7%),枕部3例(8.6%)。經(jīng)過1~6年的隨訪,15例單側(cè)畸形合并癲癇患兒中有3例耐藥(20.0%),控制無發(fā)作的患兒有12例(80.0%);3例雙側(cè)畸形合并癲癇患兒全部耐藥(100.0%),耐藥性在單雙側(cè)腦裂畸形的差異比較具有統(tǒng)計學(xué)意義(P=0.025)。結(jié)論腦裂畸形是一種少見的大腦皮質(zhì)發(fā)育的結(jié)構(gòu)障礙性疾病,雙側(cè)腦裂畸形患兒臨床表現(xiàn)嚴(yán)重,常出現(xiàn)運動發(fā)育嚴(yán)重缺陷,智力及語言發(fā)育障礙;癲癇發(fā)生率低,一旦發(fā)生往往成為藥物難治性癲癇。單側(cè)腦裂畸形患兒癲癇多數(shù)用抗癲癇藥物發(fā)作可控制。腦裂畸形伴裂隙外其他部位皮質(zhì)發(fā)育不良(裂隙以外同側(cè)半球巨腦回、小腦巨腦回,對側(cè)半球?qū)ΨQ位置多微腦回、灰質(zhì)異位,室管膜結(jié)節(jié)性灰質(zhì)移位等)以及開唇型腦裂畸形更易導(dǎo)致患兒癲癇發(fā)作。
[Abstract]:Objective to summarize the relationship between clinical manifestations and imaging features in children with cleft cerebral malformation, and to investigate the difference of prognosis in order to provide reference for diagnosis and prognosis of cleft cerebral deformity. Methods from January 2009 to May 2015, 35 children with cerebral fissure malformation were analyzed and followed up. According to the characteristics of cranial magnetic resonance imaging (MRI), 35 children with cerebral fissure deformity were divided into two groups. The clinical features of bilateral cerebral fissure deformity were compared. Results among the 35 cases of cleft malformation, 19 cases were male and 16 cases were female, 24 cases had unilateral cleft malformation, including 16 cases of hemiplegia and 66.7%, 8 cases of no motor disorder, 8 cases of cognitive development defect, and 6 cases of language disorder. There were 11 cases with bilateral cleft malformation, 5 cases with quadriplegia, 4 cases with hemiplegia, 36.4 cases with hemiplegia, 2 cases with no motor disorder, 9 cases with cognitive impairment, 81.8 cases with cognitive impairment, and 9 cases with language disorder. There were significant differences in motor developmental defects, cognitive developmental defects and language disorders between unilateral and bilateral cerebral fissure deformities (P < 0. 002, P < 0. 012 or P < 0. 003). The binary Logistic regression analysis showed that the main risk factors of epileptic seizure were hypoplasia of cortex outside the fissure (4.44% CI: 1.57-12.57) and open lip cleft malformation (P0.029 CI: 1.11-6.74). The imaging features were as follows: obturator type in 10 cases, open labial type in 21 cases, open lip type in 21 cases, mixed type in 4 cases, the fissure was located in the frontal part in 19 cases, in the top part in 11 cases, in the top part of the forehead in 11 cases, in 5. 7% in the top part, and in the occipital part in 3 cases, in the occipital part in 3 cases. After 1 ~ 6 years follow-up, 3 of 15 patients with unilateral deformity complicated with epilepsy were found to be drug-resistant 20.0%, and 12 of them without seizure control were found to have all drug resistance of 100.00.The drug resistance in unilateral cerebral fissure malformation was different in 3 patients with bilateral deformities and epilepsy. There was statistical significance in P0. 025 (P < 0. 025). Conclusion the deformity of cerebral fissure is a rare structural disorder of cerebral cortex development. The clinical manifestation of bilateral cleft deformity is serious, the motor development is serious, the intelligence and language development are impaired, and the incidence of epilepsy is low. Once it happens, it often becomes a drug-resistant epilepsy. Epilepsy in children with unilateral cleft deformity is controlled by antiepileptic drugs. Cleft deformities accompanied with dysplasia of the cortex in other parts of the fissure (except the ipsilateral megalencephalon gyrus, cerebellar megagrangial gyrus, contralateral hemispheric symmetrical multiple microgyrus, gray matter heterotopic), Ependymal nodular gray matter displacement) and open lip cleft deformity are more likely to lead to epilepsy in children.
【學(xué)位授予單位】：鄭州大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R742

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