作者@article {Cerulli Irellie1865 = {Emanuele Cerulli Irelli Enrico Cocchi和格鲁吉亚Ramantani罗伯托·h·Caraballo Loretta朱利亚诺和了图雷Yilmaz亚历山德拉Morano和Eleni Panagiotakaki弗朗西斯卡·Operto和Beatriz冈萨雷斯吉拉尔德茨Katri Silvennoinen莎拉Casciato和马里昂Comajuan西蒙娜Balestrini和弗朗西斯科·Fortunato Antonietta科波拉吉安卡洛迪詹纳罗和安吉洛Labate维托索非亚,格哈德•j•克鲁格和Doroth {} \ ' e e G.A. Kasteleijn-Nolst Trenit {\ ' e}和安东尼奥Gambardella Betul Baykan和桑杰m . Sisodiya和Alexis Arzimanoglou Pasquale Striano和卡洛迪特兰西代表EMA研究小组},title = {Electroclinical特性和长期在患者眼睑肌阵挛癫痫预后缺席},体积={98}={18},页面= {e1865——e1876} = {2022}, doi = {10.1212 / WNL。出版商0000000000200165}= {Wolters Kluwer健康,公司代表美国神经病学学会},文摘={背景和目标眼睑肌阵挛(EM)缺席(EMA)是一种全身性癫痫综合征预后和临床特点首页仍部分定义。我们调查electroclinical表型和长期癫痫预后患者群体的教育津贴。在这个多中心回顾性研究方法,EMA患者> = 5年的随访。我们调查预测模式和终端持续缓解(STR),连同他们的预后因素。此外,两步聚类分析被用来研究不同EMA表型的存在。结果包括172名患者的发病年龄平均7年(四分位距(差)5 {\ textendash} 10年)和平均随访时间14年(IQR 8.25 {\ textendash} 23.75年)。六十六名患者(38.4 \ %)显示nonremission模式,而缓解和复发模式中遇到56(32.6 \ %)和50(29.1 \ %)的参与者。早期癫痫发作,发热性癫痫(FS)的历史,EM癫痫持续状态显著预测nonremission模式根据多项逻辑回归分析。STR实现到68年(39.5 \ %)患者平均14.05年的延迟(SD {\ textpm} 12.47年)。早期癫痫发作、精神并存状况和历史的FS和广义tonic-clonic癫痫相关概率较低的实现根据Cox回归STR比例风险模型。抗癫痫药物(ASM)撤军是企图在62年172例,癫痫复发74.2 \ %。 Cluster analysis revealed 2 distinct clusters with 86 patients each. Cluster 2, which we defined as EMA-plus, was characterized by an earlier age at epilepsy onset, higher rate of intellectual disability, EM status epilepticus, generalized paroxysmal fast activity, self-induced seizures, FS, and poor ASM response, whereas cluster 1, the EMA-only cluster, was characterized by a higher rate of seizure remission and more favorable neuropsychiatric outcome.Discussion Early epilepsy onset was the most relevant prognostic factor for poor treatment response. A long latency between epilepsy onset and ASM response was observed, suggesting the effect of age-related brain changes in EMA remission. Last, our cluster analysis showed a clear-cut distinction of patients with EMA into an EMA-plus insidious subphenotype and an EMA-only benign cluster that strongly differed in terms of remission rates and cognitive outcomes.ASM=antiseizure medication; ECS=eye closure sensitivity; EM=eyelid myoclonia; EMA=EM with absences; FS=febrile seizures; GGE=genetic generalized epilepsies; GPFA=generalized paroxysmal fast activity; GTCS=generalized tonic-clonic seizures; HR=hazard ratio; ID=intellectual disability; IQR=interquartile range; JME=juvenile myoclonic epilepsy; LEV=levetiracetam; LTG=lamotrigine; OR=odds ratio; PS=photosensitivity; PWD=polyspike-wave discharges; STR=sustained terminal remission; SWD=spike-wave discharge; TSCA=2-step cluster analysis; VPA=valproate}, issn = {0028-3878}, URL = {//www.ez-admanager.com/content/98/18/e1865}, eprint = {//www.ez-admanager.com/content/98/18/e1865.full.pdf}, journal = {Neurology} }