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find Keyword "婴儿痉挛" 14 results
  • 神经炎症在婴儿痉挛发病机制中的研究进展

    婴儿痉挛(Infantile spasms,IS)是一种独特的,并有年龄依赖性的婴儿早期癫痫性脑病。具有发病年龄早、发作形式特殊、进行性认知损害、脑电图呈高峰失律等特征。其病因复杂、多样,发病机制尚未明确,治疗方面仍存在困难,大多遗留智能缺陷等后遗症。癫痫的发生与神经组织微环境中增加的强烈而持续的炎症状态相关,受损神经元组织中炎症细胞和分子的激活、分解调节障碍是癫痫发展的关键因素,炎症可能起源于中枢神经系统,或通过血脑屏障的破坏从全身循环获得。同时癫痫也可能激活促炎通路,导致神经炎症的发生。本文对近年神经炎症通路在 IS 发病机制中的作用研究进行综述,通过总结遗传学进展揭示了许多参与 IS 发病机制的基因,包括直接或间接参与炎症的基因,同时得到临床和 IS 动物模型的研究支持。了解 IS 发生发展过程中炎症的神经生物学将有助于开发新的生物标志物,以便更好地筛选高危患者,为探索 IS 治疗新靶点提供方向。

    Release date:2020-05-19 01:07 Export PDF Favorites Scan
  • The advantage of diffusion weighted imagin technique in the toxicity detection of vigabatrin

    ObjectiveTo explore the differences in the detection of vigabatrin-associated brain abnormalities on MRI by different MRI sequences, so as to further guide the clinical understanding of VABAM and improve the appropriate imaging sequences. MethodsA total of 353 patients with infantile spasm or epileptic spasm who were admitted to the Epilepsy Center of Yuquan Hospital of Tsinghua University from January 2020 to January 2023 were retrospectively included. MRI was performed in 131 cases, including 3D T1, T2, T1- fluid-attenuated inversion recovery sequence (FLAIR) images, DWI and ADC sequences, of which 65 cases taking VGB. We aim to evaluate the detection of vigabatrin-associated brain abnormalities on MRI by different MRI sequences in these children. Results Among the 65 patients, VABAM was detected in 23 cases, the detection rate was 35.4%. The average dosage of vigabatrin was 100.73±35.54 mg/(kg·d). The positive detection rates of VABAM were 95.7% in DWI sequence, 26.1% in ADC sequence, 21.3% in FLAIR sequence, 4.3% in T2 sequence and 0 in T1 sequence. The detection rate of ADC sequence was significantly different from DWI sequence and T1 sequence, but not from T2 sequence and FLAIR group. ConclusionDWI sequence has irreplaceable advantages in the detection rate of VABAM. Therefore, for patients with infantile spasm and epileptic spasm who take vigabatrin, we should try our best to add DWI sequence scanning to improve the positive detection rate and avoid clinical symptoms, so as to avoid further brain damage.

    Release date:2024-01-02 04:10 Export PDF Favorites Scan
  • Analysis of the causes of infant spasm

    ObjectiveTo analysis the causes of infant spasm (IS) and provide a theoretical basis for clinical diagnosis and treatment. MethodsCollected the clinical data of 116 IS cases in our hospital from May 2011 to December 2013, which conform to the diagnostic standard, and analysis its causes. ResultsIn the116 cases we collected, symptomatic IS is primarily for 78 cases (67.24%); the onset age of symptomatic IS within 6 months was 76.39% (55/72), higher than the rates of symptomatic IS beyond 6 months 52.27% (23/44) (P=0.007); The pathogenic factor in symptomatic IS, prenatal accounted for 48.72%, intrapartum factors accounted for 34.62%; for sexually transmitted diseases accounted for 47.44%, venereal disease accounted for 52.56%. In 78 cases of symptomatic IS, gender has no relation with its pathogenic factors; the onset age within 6 months of antepartum and intrapartum factor is significantly higher than the rates of symptomatic IS beyond 6 months (89.09% VS 69.57%); distribution between urban and rural areas and its pathogenic factors is related, prenatal factors of urban IS significantly lower than which in rural areas (38.30% VS 64.51%), intrapartum factor IS significantly higher than the rural IS (44.68% VS 19.35%). ConclusionThe cause of the IS given priority to with symptomatic, IS in the majority with, and prenatal or developmental factors in the higher flight in symptomatic, and the smaller of the onset age antepartum and intrapartum factor becomes more apparent, prenatal factors in the rural areas is higher than which in the unban areas, but intrapartum factor in the unban areas is higher than which in the rural areas. Magnetic resonance imaging (MRI) still plays an important role in etiology diagnosis.

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  • 婴儿痉挛新型药物治疗的研究进展

    婴儿痉挛是婴儿最常见的癫痫性脑病之一,早期诊断和治疗可以改善患儿神经发育的预后。促肾上腺皮质激素(Adrenocorticotropic hormone,ACTH)及氨己烯酸是目前临床首选的一线治疗方案。此外还有一些具有动物实验或临床研究价值但尚未大范围用于临床治疗的新型药物,如雷帕霉素、氟桂利嗪、非氨酯、褪黑素及大麻二酚等,为这一难治性癫痫提供了新的治疗见解。本综述阐述了婴儿痉挛新型药物治疗的最新进展及实际应用。

    Release date:2022-10-31 09:25 Export PDF Favorites Scan
  • 维生素B6反应性癫痫

    维生素B6(VitaminB6, VB6) 相关性癫痫或发作主要包括VB6缺乏性惊厥、VB6依赖性癫痫和VB6反应性癫痫,前二者由于社会和科学的进步可以被预防逐渐被人们认识并得到有效治疗,但VB6反应性癫痫尚未得到广泛重视。VB6反应性癫痫是指发作可被VB6单药控制,或在已有抗癫痫药物不能控制的基础上加用VB6后发作控制达1个月以上,治疗一定时间后可停用而不会复发。其临床特点包括:① 发作起始年龄在3个月~5岁,大多数在1岁以内;② 癫痫多为婴儿痉挛,少数为Lennox-Gastaut综合征、强直-阵挛发作或局灶运动性发作;③ 病因不仅为特发性或隐源性,也可为有器质性脑损伤的症状性;④ 色氨酸负荷试验通常为阴性;⑤ 口服大剂量VB6可使发作减少或消失;⑥ 排除VB6缺乏性惊厥和VB6依赖性癫痫。VB6反应性癫痫的发病机制尚不清楚,可能与年龄依赖的酶功能异常,或与VB6依赖性神经递质的功能成熟关键阶段有关,预后良好。

    Release date:2017-05-24 05:46 Export PDF Favorites Scan
  • Expression of corticotropin releasing hormone and its receptors in infantile spasm

    ObjectiveThe purpose of this study was to explore the expression of Corticotropin releasing hormone (CRH), Corticotropin releasing hormone receptor 1 (CRHR1), Protein kinase C (PKC) in epileptogenic zone of Infantile spasm (IS).MethodsCollected 17 cases of tissues of IS patients from operation and 6 cases of normal brain tissues from clinical autopsy during June 2011 to June 2014. Westen blot was used to detected the protein expression of CRH, CRHR1, PKC. PCR was used to exam the mRNA expression of CRH, CRHR1, PKC. Immunohistochemistry and fluorescenceimmuno assay were used to detect the expression of CRH, CRHR1, PKC.ResultsThe mRNA expression of CRH and CRHR1 in IS group are higher than control group, and the protein expression of CRH and CRHR1 in IS group are higher than control group. CRH are slightly expressed in the controls, medium and strong expressed in IS, CRH and NF200 both expressed in IS; CRH is negative in GFAP positive astrocyte; CRH is negative in HLA positive microglial cell. CRHR1 are slightly and medium expressed in the controls, medium and strong expressed in IS, CRHR1 and NF200 both expressed in IS; CRHR1 and GFAP are both positive in astrocyte; CRHR1 and HLA are both positive in microglial cell. PKC are slightly and medium expressed in the controls, medium and strong expressed in IS, PKC and NF200 both expressed in IS; PKC and GFAP are both positive in astrocyte; PKC and HLA are both positive in microglial cell. Spearman analysis showed positive correlation between the expression of CRH, CRHR1, PKC with epileptic spasm in IS patients, as well as positive correlation between PKC with CRHR1.ConclusionsOver expression of CRH, CRHR1, PKC with epileptic spasm in IS patients were positive related with epileptic seizure in IS patients, indicated that CRH signal pathway is related with IS pathogenesis.

    Release date:2017-01-22 09:09 Export PDF Favorites Scan
  • COL4A1基因变异致伴或不伴眼部异常的脑小血管病1型一例

    Release date:2023-09-07 11:00 Export PDF Favorites Scan
  • 婴儿痉挛症动物模型研究进展及其评价

    婴儿痉挛症(Infantile spasm,IS)是一种婴幼儿期难治性癫痫性脑病,临床表现是点头抱团样痉挛发作,脑电图(EEG)呈发作间期高度失律以及精神运动发育落后。大多数患儿促肾上腺皮质激素(Adrenocorticotropic Hormone,ACTH)和氨己烯酸(Vigabatrin,VGB)治疗有效,对普通抗癫痫药物效果不佳。婴儿痉挛症的病因有 200 多种,但至今发病机制不明。本文总结了 7 个有关婴儿痉挛症典型的动物模型。ARX 基因突变小鼠模型对雌二醇治疗有效,且提出了中间神经元致病学说。唐氏综合症小鼠模型由氨基丁酸 B 受体(GABABR)激动剂诱发痉挛,对托肽品 Q 治疗有效。N-甲基-D-天冬氨酸(NMDA)可以诱导大、小鼠痉挛发作,在产前给予倍他米松或是产前游泳模拟产前压力解释了 ACTH 治疗有效性的原理。多重打击大鼠模型模拟大脑皮层受损导致的癫痫,做出了耐药模型。河豚毒大鼠模型是唯一有 EEG 发作间期高度失律的模型,并且和“发作不同步学说”相一致。本文回顾学习 7 个婴儿痉挛症不同动物模型的特点和局限性,探讨婴儿痉挛症的发病机制,以及部分模型的新药研究。

    Release date:2019-03-21 11:04 Export PDF Favorites Scan
  • The effect of the mode of continuous guidance with wechat group on the ketogenic diet retention rate in children with infantile spasms

    ObjectiveTo explore the impact on the retention and effective rate of the mode of continuous guidance with wechat group in children with infantile spasms (IS) treated by ketogenic diet (KD).MethodsThe clinical data of 40 children who received KD treatment in Jiangxi Children’s Hospital from January 2017 to June 2019 were retrospectively analyzed, including 23 males and 17 females, the average age was (19.90±13.10) months and the average course was (9.95±7.61) months. They were randomly divided into control group (20 cases) and observation group (wechat group continuity Guidance Group, 20 cases), and followed up one year to compare the retention rate and efficacy.ResultsThere was no statistical difference between the general data of the observation group and the control group (P>0.05). After 9 and 12 months of KD treatment, the retention and effective rate of the observation group was significantly higher than the control group (P<0.05). And the seizure-free rate was higher than the control group (35% vs. 10%, 35% vs. 15%).ConclusionThis model of continuous guidance with wechat group can increase the KD retention rate of IS children, And increase the effective rate.

    Release date:2021-04-25 09:50 Export PDF Favorites Scan
  • A long-term follow-up prognostic analysis of infantile spasm

    ObjectiveTo understand the prognosis of infantile spasm through long-term follow-up. MethodsChildren with infantile spasm diagnosed and treated in Children’s Hospital from January 2010 to December 2015 were retrospectively analyzed. Clinical data were collected and telephone follow-up was conducted. ResultsA total of 169 cases were collected, and only 59 cases were successfully followed up, of which 13 patients were allowed to attend school. 38 patients were not allowed to go to school, 8 patients were dead. In the group that did not go to school, 17 of them could not walk, accounting for 44.74% of the non-school attendance group, 27 of them could not run, accounting for 71.05% of the non-school attendance group. There are 33 caese who could only depend on family care instead of themselves, accounting for 86.84% of the non-school group.There were 36 children who could not speak simple sentences, accounting for about 94.74%. In addition, in terms of seizures, there were 15 patients seizure-free for more than 5 years, accounting for 29.41% of the survival group. ConclusionThe long-term prognosis of infantile spasm was poor, the mortality rate was 13.56%, the school attendance rate was 25.5% in the survival group, and the self-care ability of the non-school attendance group was poor. The normal development at the onset of the disease and the early control of epilepsy without seizures are the important prognostic factors of the disease.

    Release date:2022-10-31 09:25 Export PDF Favorites Scan
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