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find Keyword "肌阵挛" 13 results
  • Efficacy of Pretreatment of Vecuronium Combined with Dilution of Etomidate on Etomidateinduced Myoclonus

    目的:旨在评价预注维库溴铵联合稀释依托咪酯减轻依托咪酯全麻诱导中肌阵挛的效果。方法:本研究为前瞻性、双盲、随机对照研究。80名ASA I-II级、年龄18~60岁、拟行胆囊切除术的全麻患者被随机分为4组:组1,预注维库溴铵0.01 mg/kg;组2,依托咪酯用生理盐水由2 mg/mL 稀释为1 mg/mL;组3,预注维库溴铵联合稀释依托咪酯;组4,生理盐水对照组+非稀释依托咪酯组。根据分组给予患者预注维库溴铵0.01 mg/kg或同等量的生理盐水,观察并询问患者有无呼吸困难,并记录呼吸频率和脉搏氧饱和度(SpO2)。3分钟后推注稀释或无稀释依托咪酯0.3 mg/kg,询问患者有无注射痛并做疼痛评分,观察有无肌阵挛并评估其程度。2分钟后给予维库溴铵0.1 mg/kg、芬太尼3 μg/kg和丙泊酚1 mg/kg行气管插管。实验期间同时记录无创动脉血压(BP)和心率(HR)。结果:组1和组4分别有2例(10%)和3例(15%)患者述轻度注射疼痛,而组2和组3无患者述注射疼痛。组1、组2和组3肌阵挛的发生率明显低于组4(30%、40%和15% vs 70%,Plt;0.05)。且组1、组2和组3肌阵挛的程度多为轻中度,而组4多为中重度。四组患者均未述任何呼吸困难,呼吸频率无明显降低,SpO2无明显变化。四组患者BP和HR变化一致,无明显差别。结论:预注小剂量维库溴铵或稀释依托咪酯可明显降低依托咪酯引起肌阵挛的发生率并减轻其程度。且这两种方法联合应用比单独应用效果更佳,具有一定程度的协同作用。

    Release date:2016-09-08 10:02 Export PDF Favorites Scan
  • Clinical electrophysiological features and efficacy of anti-epileptic drugs of patients with Juvenile myoclonic epilepsy

    ObjectiveTo summarize clinical electrophysiological features and efficacy of some of Anti-epileptic drugs(AEDs) of Juvenile myoclonic epilepsy (JME). MethodsClinical electrophysiological information of 101 outpatients with JME observed at Xuanwu Hospital from Jul. 2001 to Sep. 2014 was retrospectively analyzed, including the seizure types, trigger factors, electroencephalogram. We followed some of these patients and compared the efficacy between different AEDs. Result According to different seizure types, there are four subtypes: Myoclonus (MJ) only 11.88%, MJ+generalized tonic-clonic seizure(GTCS) 75.24%, MJ+GTCS+Absence(Abs) 11.88%, MJ+Abs 1.00%. Patients with typical ictal generalized poly-spike and waves (PSW) or spike and waves (SW) or spikes account for 96.80%. And 75.00% of patients have no MJ and 91.80% have no GTCS with valproic acid monotherapy. 65.00% and 88.24% of patients were seizure free of MJ and GTCS recpectively. But the difference of efficacy between these two drugs have no statistically significance. Sleep deprivation was the primary trigger factors, accounting for 16.83%. ConclusionJME has clinical heterogeinety, clinicians should fully understand the whole condition of JME individual, including their clinical manifestation, EEG features, reaction to AEDs, trigger factors, habitual patterns and so on, in order to help making individualized therapy.

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  • 青少年肌阵挛癫痫基因的研究

    青少年肌阵挛癫痫(Juvenile myoclonic epilepsy, JME)是特发性癫痫中常见的癫痫综合征, 有明显的遗传和表型的异质性。遗传因素在JME发病中起重要作用, 随着JME相关基因不断被发现, JME在分子层面的发病机制也在不断进展, 基因型与表现型的关系也在进一步研究。目前发现的与青少年肌阵挛癫痫相关的基因有:CACNB4、GABRa1、GABRD、EFHC1、CASR、CPA6、BRD2、Cx-36、ME2。文章主要总结JME基因及其致病机制, 同时介绍基因型和表型关系的研究进展

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  • Clinical and electroencephalogram features of dyssynergia cerebellaris myoclonica

    ObjectiveWe report two family and one sporadic case with dyssynergia cerebellaris myoclonica, investigate the clinical and neural electrophysiological features. MethodsThe proband and sporadic patient was examined by clinical, neuroimaging, video-EEG and synchronous electromyography. ResultsThere were 6 patients with dyssynergia cerebellaris myoclonica of the 27 family members in the first family(3 male and 3 female). There were 4 patients with dyssynergia cerebellaris myoclonica of the 20 family members in the second family(2 male and 2 female). All patiens had disproportionately myoclonus, epilepsy and progressive cerebellar ataxia. EEG showed bursts of spike-slow wave, polyspilke-slow wave distributing in the bilateral brain both in ictal and interictal period, sometimes it is especially in central, parietal and frontal area. EEG showed bursts of spike-slow wave, polyspilke-slow wave distributing in the central, parietal and frontal area in interictal period. Pathology of the skin and muscles are normal. ConclusionThe diagnosis of dyssynergia cerebellaris myoclonica was mainly based on typical clinical manifestations, brain MRI and EEG changes.Long time video EEG and synchronous EMG is important for the diagnosis. Skin and muscles pathology can be normal.

    Release date:2016-10-02 06:51 Export PDF Favorites Scan
  • Efficacy and Safety of Midazolam in the Prevention of Etomidate-induced Myoclonus: A Meta-analysis

    ObjectiveTo evaluate the efficacy and safety of midazolam in the prevention of etomidate-induced myoclonus. MethodsDatabases including PubMed, EMbase, The Cochrane Library (Issue 8, 2015), CBM, WanFang Data, VIP, and CNKI were electronically searched to collect randomized controlled trials (RCTs) about midazolam in the prevention of etomidate-induced myoclonus from inception to August, 2015. Two reviewers independently screened literature, extracted data and assessed the risk of bias of included studies. Then, meta-analysis was performed by RevMan 5.2 and Stata 12.0 softwares. ResultsA total of 14 RCTs involving 1 274 patients were included. The results of metaanalysis showed that, compared with placebo, pretreatment with midazolam injection could reduce the incidence of myoclonus (RR=0.28, 95%CI 0.19 to 0.42, P<0.000 01). The sub-group analysis based on different doses of midazolam showed that all three different doses of midazolam (0.015 mg/kg, 0.03 mg/kg and 0.05-0.1 mg/kg) could reduce the incidence of myoclonus effectively (all P values <0.05). ConclusionPretreatment with midazolam injection can reduce the incidence of etomidate-induced myoclonus without increasing the risk of recovery latency and over sedation. Due to the limited quality of included studies, the above conclusion needs to be further verified by more high quality studies.

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  • I.V. Infusion of Dezocine before Etomidate Administration for Myoclonus of Prevention Caused by Etomidate: A Systematic Review

    ObjectiveTo systematically assess the effectiveness and safety of I.V. infusion of dezocine for prevention of myoclonus caused by etomidate. MethodsDatabases including PubMed, EMbase, The Cochrane Library (Issue 6, 2014), CNKI, WanFang Data and VIP were electronically searched from inception to May 2014 for randomized controlled trials (RCTs) on I.V. infusion of dezocine for prevention of myoclonus caused by etomidate. Two reviewers independently screened literature, extracted data, and assessed the risk of bias of included studies. Then, meta-analysis was performed using RevMan 5.2.3 software. ResultsTen RCTs were included. The results of meta-analysis indicated that, dezocine could reduce the incidence of myoclonus induced by etomidate (RR=0.24,95%CI 0.12 to 0.45, P<0.000 1), and was better than fentanyl (RR=0.30, 95%CI 0.17 to 0.51, P<0.000 1); dezocine could reduce the amount of etomidate (MD=-4.70, 95%CI -6.62 to -2.79, P<0.000 01); compared with fentanyl, dezocine could reduce the incidence of injection pain (OR=0.25, 95%CI 0.10 to 0.62, P=0.003); dezocine did not increase the incidence of respiratory depression (OR=2.61, 95%CI 0.12 to 56.03, P=0.54). ConclusionI.V. infusion of dezocine before etomidate administration could reduce myoclonus incidence caused by etomidate, reduce the amount of etomidate, and is better than fentanyl; which could also reduce the incidence of injection pain, and not increase the incidence of respiratory depression.

    Release date:2016-10-02 04:54 Export PDF Favorites Scan
  • Clinical study about the patients onset epilepsy diaginose of Dentatorubral-pallidoluysian atropy

    ObjectiveTo study the clinical characteristics of patients onset epilepsy Dentatorubral-pallidoluysian atropy (DRPLA) in Epilepsy Center of Guangdong 999 Brain Hospital and improve understanding of the disease. MethodsCollected five patients from August 2014 to August 2016 in Guangdong 999 Brain Hospital, whom diagnosed through genetic testing of DRPLA, analysed their disease course, family history, video-EEG, brain MRI and treatment data. ResultsDRPLA performed as neurodegenerative diseases, and epilepsy population mainly performed as progressive myoclonic epilepsy (Progressive myoclonus epilepsy, PME). ConclusionDRPLA is autosomal dominant neurodegenerative disease. In patients with cerebellar atrophy, neurological regression, ataxia, drug refractory epilepsy, it is recommended routinely to detect ATN1 gene, so that timely diagnosis and genetic counseling.

    Release date:2016-11-28 01:27 Export PDF Favorites Scan
  • The clinical features and Video-EEG of Eyelid myoclonia-nonconvulsive status epilepticus in children

    ObjectiveTo study the clinical and EEG features, therapeutic response and prognosis of eyelid myoclonia-nonconvulsive status epilepticus (EM-NCSE) in children.MethodsCollected the clinical and EEG data of 3 children with EM-NCSE that were diagnosed in department of neurology in Qilu Children Hospital of Shandong university during the January in 2015 to August in 2016.Analysed the therapeutic response to antiepletic drugs(AEDs).ResultsAmong the three children, there were 2 girls and 1 boy.The age at the onset of the disease was from 6 to 10 years old.The average age of them is 8.67 years old.The clinical manifestations include mental confusion, dysphoria, winking and scrolling up the eyes.The typical vedio electroencephalography (VEEG) in the patients showed 3~6 Hz generalized spike and waves and polyspikes burst, especially in the frontal and the anterior temporal region.In addition, the eye closure and intermittent photic stimulation helped to induce discharges and clinical events as eyelid myoclonia (EM).ConclusionsEM-NCSE is one of the idiopathic and generalized epileptic disease and characterized by EM.Video EEG monitoring plays an important role in the diagnosis of this disease.The drugs of choice for treatment was diazepam.When the event was controlled, AEDs were effective for the following therapy.

    Release date:2017-05-24 05:46 Export PDF Favorites Scan
  • 肌阵挛与肌阵挛癫痫

    肌阵挛是中枢神经系统所致突然、短暂、电击状(shock like)不随意运动。肌阵挛癫痫定义尚有争议,目前认为可能是涵盖癫痫的电临床意义,累及下行的神经元,此神经元的扩展或颞叶扩充能扳击为明显的痫性活动。肌阵挛可能是多种癫痫综合征的一部分(如青少年肌阵挛、全面强直-阵挛或进行性肌阵挛癫痫),也可能是单独发作表现(如良性肌阵挛癫痫),或是多种癫痫类型之一(如肌阵挛性-不稳定性癫痫)。肌阵挛癫痫分为皮层性肌阵挛、丘脑-皮层性肌阵挛、皮层反射性肌阵挛、阴性肌阵挛、良性家族性皮质性肌阵挛震颤癫痫、进行性肌阵挛癫痫、青少年肌阵挛癫痫。一般传统抗癫痫药物,除丙戊酸、氯硝基安定、苯巴比妥外,其他抗癫痫药物均无效。新型抗癫痫药物已证实左乙拉西坦、托吡酯、唑尼沙胺、吡拉西坦及吡仑帕奈有效,但对不同类型肌阵挛癫痫治疗效果有差异。

    Release date:2021-06-24 01:26 Export PDF Favorites Scan
  • 肌阵挛性失张力癫痫的表型与遗传谱

    文章旨在描述一个大样本量的癫痫伴肌阵挛性失张力发作(MAE)患者神经发育损害程度,并确定其遗传学病因。采用标准化的神经心理学仪器对MAE患者的癫痫特征、智力残疾、自闭症谱系障碍和注意缺陷/多动障碍进行深入的表型分析。我们对癫痫和神经精神疾病的基因集进行外显子分析(全外显子测序),以确定遗传学病因。研究共分析了101例MAE患者(70%为男性)。发作年龄中位数为34月龄(范围6~72月龄)。主要发作类型为肌阵挛性失张力发作或失张力发作100%、全身强直阵挛性发作72%、肌阵挛性发作69%、失神性发作60%、强直性发作19%。研究观察到62%的患者有智力障碍,69%的患者适应行为评分极低。此外,24%表现出自闭症症状,37%表现出注意力缺陷/多动症状。85例患者中的12例(14%)发现了致病性变异,包括5例先前发表的患者。这些基因是SYNGAP1(n=3)、KIAA2022(n=2)、SLC6A1(n=2)以及KCNNA2、SCN2A、STX1B、KCNB1和MECP2(各n=1)。此外,研究还分别在3例患者中分别鉴定了1个新的候选基因—ASH1L、CHD4和SMARCA2。研究发现,MAE与明显的神经发育障碍有关。MAE具有遗传异质性,通过外显子分析在14%的队列中确定了致病的遗传病因。这些发现表明MAE是几种病因的表现,而不是一个离散的综合征实体。

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