TY -的T1 Electroclinical特性和长期在患者眼睑肌阵挛癫痫预后缺席JF -神经学乔-神经病学SP - e1865 LP - e1876做- 10.1212 / WNL。首页0000000000200165六世- 98 - 18盟Emanuele Cerulli Irelli盟Enrico Cocchi AU -格鲁吉亚Ramantani盟罗伯托·h·Caraballo AU -洛雷塔朱利亚诺盟了图雷Yilmaz AU -亚历山德拉Morano AU - Eleni Panagiotakaki盟Beatriz冈萨雷斯吉拉尔德茨-弗朗西斯卡·Operto盟盟——Katri Silvennoinen AU -莎拉Casciato AU -马里昂Comajuan盟-西蒙娜Balestrini AU -弗朗西斯科·Fortunato盟Antonietta科波拉AU -吉安卡洛迪詹纳罗盟安吉洛Labate AU -维托索非亚AU -格哈德·j·克鲁格盟多罗斯G.A. Kasteleijn-Nolst Trenite AU -安东尼奥Gambardella盟Betul Baykan盟Sanjay m . Sisodiya AU - Alexis Arzimanoglou盟Pasquale Striano AU -卡洛迪特兰西盟代表EMA研究小组Y1 - 2022/05/03 UR - //www.ez-admanager.com/content/98/18/e1865.abstract N2 -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仍部分定义。首页我们调查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≥5年的随访患者包括在内。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(四分位范围(差)5 - 10年),中位随访时间14年(IQR 8.25 - -23.75年)。六十六例(38.4%)显示nonremission模式,而缓解和复发模式遇到了56个参与者(32.6%)和50例(29.1%)。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是通过68例(39.5%)患者平均14.05年的延迟(SD±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。 Antiseizure medication (ASM) withdrawal was attempted in 62 of 172 patients, and seizures recurred in 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 ER -