Cryptogenic NORSE: Its distinctive clinical features and response to immunotherapy.
Cryptogenic NORSE: Its distinctive clinical features and response to immunotherapy.
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DOI：
10.1212/nxi.0000000000000396
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发表时间：
2017-11
期刊：
影响因子：
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通讯作者：
Nishiyama K
中科院分区：
文献类型：
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作者：
Iizuka T;Kanazawa N;Kaneko J;Tominaga N;Nonoda Y;Hara A;Onozawa Y;Asari H;Hata T;Kaneko J;Yoshida K;Sugiura Y;Ugawa Y;Watanabe M;Tomita H;Kosakai A;Kaneko A;Ishima D;Kitamura E;Nishiyama K
报道隐源性新发难治性癫痫持续状态（C-NORSE）的独特临床特征及基于初始临床评估的C-NORSE评分。回顾性研究了2007年1月1日至2016年8月31日期间136例临床疑似自身免疫性脑炎患者进行神经元表面抗原自身抗体检测。11例C-NORSE患者被确定。比较32例抗nmda受体脑炎（NMDARE）患者的临床特征。11例C-NORSE患者（中位年龄27岁；7例[64%]女性）的临床结果在中位随访11个月（范围6-111个月）后进行评估。癫痫持续状态前常伴有发热（10/11[91%]）。脑mri显示对称的T2/液体衰减反转恢复高信号（8/11[73%]）和脑萎缩（9/11[82%]）。10名接受治疗的患者中只有2名对一线免疫治疗有反应，5名接受静脉注射环磷酰胺治疗的患者中有4名对治疗有反应。8例（73%）患者的长期预后较差。与32例NMDARE患者（中位年龄27岁；24例[75%]女性）相比，C-NORSE患者有更频繁的前驱热、癫痫持续状态、呼吸支持和对称的脑MRI异常，不自主运动更少，没有心理行为症状、脑脊液低克隆带或肿瘤关联，并且预后更差。C-NORSE患者的C-NORSE评分高于NMDARE患者。C-NORSE患者的临床免疫特征与NMDARE患者不同。C-NORSE评分可能有助于区分他们。一些患者可能对免疫疗法有反应。
To report the distinctive clinical features of cryptogenic new-onset refractory status epilepticus (C-NORSE) and the C-NORSE score based on initial clinical assessments. A retrospective study was conducted for 136 patients with clinically suspected autoimmune encephalitis who underwent testing for autoantibodies to neuronal surface antigens between January 1, 2007, and August 31, 2016. Eleven patients with C-NORSE were identified. Their clinical features were compared with those of 32 patients with anti-NMDA receptor encephalitis (NMDARE). The clinical outcome of 11 patients (median age, 27 years; 7 [64%] women) with C-NORSE was evaluated after a median follow-up of 11 months (range, 6–111 months). Status epilepticus was frequently preceded by fever (10/11 [91%]). Brain MRIs showed symmetric T2/fluid-attenuated inversion recovery hyperintensities (8/11 [73%]) and brain atrophy (9/11 [82%]). Only 2 of the 10 treated patients responded to the first-line immunotherapy, and 4 of the 5 patients treated with IV cyclophosphamide responded to the therapy. The long-term outcome was poor in 8 patients (73%). Compared with 32 patients with NMDARE (median age, 27 years; 24 [75%] women), those with C-NORSE had more frequent prodromal fever, status epilepticus, ventilatory support, and symmetric brain MRI abnormalities, had less frequent involuntary movements, absent psychobehavioral symptoms, CSF oligoclonal bands, or tumor association, and had a worse outcome. The C-NORSE score was higher in patients with C-NORSE than those with NMDARE. Patients with C-NORSE have a spectrum of clinical-immunological features different from those with NMDARE. The C-NORSE score may be useful for discrimination between them. Some patients could respond to immunotherapy.