Prognostic significance of subsequent extra-temporal involvement in cryptogenic new onset refractory status epilepticus (NORSE) initially diagnosed with limbic encephalitis
Prognostic significance of subsequent extra-temporal involvement in cryptogenic new onset refractory status epilepticus (NORSE) initially diagnosed with limbic encephalitis
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DOI：
10.1016/j.eplepsyres.2019.106215
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发表时间：
2019-12-01
影响因子：
2.2
通讯作者：
Huh, Kyoon
中科院分区：
文献类型：
--
作者：
Choi, Jun Young;Kim, Eun Jin;Huh, Kyoon
简介：新发难治性癫痫持续状态（NORSE）定义为无活动性癫痫或相关神经系统疾病且无明确活动性病因的难治性SE患者。在不同的人群中报道了不同类型和病因的NORSE。据报道，边缘脑炎（LE）是NORSE的病因之一。在这项研究中，我们调查了NORSE与边缘脑炎之间是否存在交叉点，以及存在交叉点患者的预后因素。方法：我们回顾性分析数据库中符合NORSE定义和LE初始诊断标准的患者。临床特点和血液检查，脑脊液，脑电图和磁共振成像结果进行了综述。预后记录为ICU住院时间、总住院时间、出院时修正Rankin量表。特别是，我们确定了哪些因素与患者预后相关。结果：入选患者13例。13例患者中有9例出现肌痛，8例在前驱期出现发热。13例患者中有12例在SE发展前有急性记忆障碍或混乱。此外，46.2%的患者出现白细胞减少或血小板减少。入院时中位体温为37.6℃，9例患者表现为全身性强直-阵挛性SE。所有患者均接受免疫治疗，13例患者中有11例通过诱导昏迷治疗实现了爆发抑制。随访影像显示病变扩大10例。最常见的延伸部位是耻骨。病变扩展较大的患者预后较无病变扩展的患者差。结论：NORSE-LE患者继发颞外病变扩大与预后不良密切相关。本研究探索了一种新的NORSE亚型，并提出了NORSE和LE之间可能的共同潜在病理机制。
Introduction: New-onset refractory status epilepticus (NORSE) is defined as refractory SE in patients without active epilepsy or relevant neurological disorder with no clear active causes. Diverse types and etiologies of NORSE are reported in various groups. Limbic encephalitis (LE) is reported as one of etiologies of NORSE. In this study, we investigated whether there were any intersections between NORSE and limbic encephalitis, as well as the presence of prognostic factors in intersection patients.Methods: We retrospectively analyzed patients who met both the definition of NORSE and diagnostic criteria of LE at the initial presentation from our database. Clinical characteristics and blood test, cerebrospinal fluid, electroencephalography, and magnetic resonance imaging results were reviewed. Prognosis was recorded as ICU admission stay, total length of hospitalization, and modified Rankin Scale at discharge. In particular, we determined which factors were associated with patients' prognosis.Results: Thirteen patients were selected. Nine of the 13 patients had myalgia and 8 patients had fever in the prodromal period. Twelve of the 13 patients had acute memory impairment or confusion before SE development. In addition, 46.2% of the patients showed leukopenia or thrombocytopenia. Median body temperature at hospital arrival was 37.6 degrees C. Nine patients showed generalized tonic-clonic SE. All patients were treated with immunotherapy and 11 of the 13 patients achieved burst suppression through induced coma therapy. Ten patients showed lesion extension on follow-up imaging. The most common extension site was the claustrum. Patients with more lesion extension showed poorer outcomes than those without lesion extension.Conclusion: Subsequent extratemporal lesion extension was closely associated with poor prognosis in NORSE-LE patients. This study explores a new subtype of NORSE and suggests a possible common underlying pathomechanism between NORSE and LE.