脑裂畸形患儿脑结构与临床及预后的对照研究

发布时间：2018-05-20 01:33

本文选题：脑裂畸形 + 癫痫　；参考：《郑州大学》2017年硕士论文

【摘要】：目的总结脑裂畸形患儿的临床表现与影像学特征之间的联系,并进行随访了解预后差异,为本病诊断及预后判断提供参考依据。方法分析2009年1月至2015年5月在河南省人民医院小儿神经内科门诊就诊的35例脑裂畸形患儿资料,并进行随访,根据头颅磁共振(MRI)特征进行分组(单、双侧脑裂畸形),比较临床特征。结果35例脑裂畸形患儿中,男19例(54.3%),女16例(45.7%);单侧脑裂畸形24例(68.6%),其中偏瘫16例(66.7%),无运动障碍8例(33.3%);认知发育缺陷8例(33.3%),语言障碍6例(25.0%)。双侧脑裂畸形11例(31.4%),其中四肢瘫5例(45.4%),偏瘫4例(36.4%),无运动障碍2例(18.2%);认知发育缺陷9例(81.8%),语言障碍9例(81.8%)。比较运动发育缺陷程度、认知发育缺陷及语言障碍的有无在单、双侧脑裂畸形的差异有统计学意义(P=0.002,P=0.012,P=0.003)。癫痫发作18例(51.4%),二元Logistic回归分析示:伴裂隙以外部位皮质发育不良(P=0.005,OR=4.44,CI:1.57-12.57)、开唇型脑裂畸形(P=0.029,OR=2.73,CI:1.11-6.74)是癫痫发作的主要危险因素。影像学特征:闭唇型10例(28.6%),开唇性21例(60.0%),混合型4例(11.4%);裂隙位于额部19例(54.3%),额顶部11例(31.4%),顶部2例(5.7%),枕部3例(8.6%)。经过1~6年的随访,15例单侧畸形合并癫痫患儿中有3例耐药(20.0%),控制无发作的患儿有12例(80.0%);3例双侧畸形合并癫痫患儿全部耐药(100.0%),耐药性在单双侧脑裂畸形的差异比较具有统计学意义(P=0.025)。结论脑裂畸形是一种少见的大脑皮质发育的结构障碍性疾病,双侧脑裂畸形患儿临床表现严重,常出现运动发育严重缺陷,智力及语言发育障碍;癫痫发生率低,一旦发生往往成为药物难治性癫痫。单侧脑裂畸形患儿癫痫多数用抗癫痫药物发作可控制。脑裂畸形伴裂隙外其他部位皮质发育不良(裂隙以外同侧半球巨脑回、小脑巨脑回,对侧半球对称位置多微脑回、灰质异位,室管膜结节性灰质移位等)以及开唇型脑裂畸形更易导致患儿癫痫发作。
[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.
【学位授予单位】：郑州大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R742

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