Department of Brain Science, Ajou University School of Medicine, Suwon, Republic of Korea; Department of Neurology, Ajou University School of Medicine, Suwon, Republic of Korea.
Department of Radiology, Ajou University School of Medicine, Suwon, Republic of Korea.
Epilepsy Res. 2019 Dec;158:106215. doi: 10.1016/j.eplepsyres.2019.106215. Epub 2019 Oct 12.
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.
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.
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 °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.
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.
新起难治性癫痫持续状态（NORSE）定义为无活动性癫痫或相关神经障碍的患者出现难治性癫痫持续状态，且无明确的活动性病因。不同类型和病因的 NORSE 在不同人群中有报道。边缘性脑炎（LE）被报道为 NORSE 的病因之一。在本研究中，我们调查了 NORSE 和边缘性脑炎之间是否存在任何交集，以及交集患者是否存在预后因素。
我们回顾性分析了数据库中符合 NORSE 定义和 LE 诊断标准的患者。回顾了临床特征和血液检查、脑脊液、脑电图和磁共振成像结果。预后记录为 ICU 入住时间、总住院时间和出院时改良 Rankin 量表评分。特别是，我们确定了哪些因素与患者的预后相关。
选择了 13 名患者。9 名患者在前驱期有肌痛，8 名患者有发热。13 名患者中有 12 名在癫痫持续状态发作前有急性记忆障碍或意识混乱。此外，46.2%的患者有白细胞减少或血小板减少。入院时的中位体温为 37.6°C。9 名患者表现为全面强直阵挛性癫痫持续状态。所有患者均接受免疫治疗，13 名患者中有 11 名通过诱导昏迷治疗达到爆发抑制。10 名患者在随访影像学上显示病变延伸。最常见的延伸部位是屏状核。病变延伸较多的患者预后较差。
NORSE-LE 患者随后出现的颞外病变延伸与预后不良密切相关。本研究探讨了一种新的 NORSE 亚型，并提示 NORSE 和 LE 之间可能存在共同的潜在发病机制。
