Cryptogenic NORSE Its distinctive clinical features and response to immunotherapy

被引：73

作者：

Iizuka, Takahiro ^{[1
]}

Kanazawa, Naomi ^{[1
]}

Kaneko, Juntaro ^{[1
]}

Tominaga, Naomi ^{[1
]}

Nonoda, Yutaka ^{[2
]}

Hara, Atsuko ^{[3
]}

Onozawa, Yuya ^{[4
]}

Asari, Hiroki ^{[5
]}

Hata, Takashi ^{[5
]}

Kaneko, Junya ^{[6
]}

Yoshida, Kenji ^{[7
]}

Sugiura, Yoshihiro ^{[7
]}

Ugawa, Yoshikazu ^{[7
,8
]}

Watanabe, Masashi ^{[9
]}

Tomita, Hitomi ^{[9
]}

Kosakai, Arifumi ^{[10
]}

Kaneko, Atsushi ^{[1
]}

Ishima, Daisuke ^{[1
]}

Kitamura, Eiji ^{[1
]}

Nishiyama, Kazutoshi ^{[1
]}

机构：

[1] Kitasato Univ, Sch Med, Dept Neurol, Tokyo, Japan

[2] Kitasato Univ, Sch Med, Dept Pediat, Tokyo, Japan

[3] Kitasato Univ, Sch Med, Dept Pathol, Tokyo, Japan

[4] Kitasato Univ Hosp, Dept Clin Lab, Sagamihara, Kanagawa, Japan

[5] Shizuoka City Shimizu Hosp, Dept Neurol, Shizuoka, Japan

[6] Tama Nagayama Hosp, Nippon Med Sch, Dept Emergency & Crit Care Med, Tama, Japan

[7] Fukushima Med Hosp, Sch Med, Dept Neurol, Fukushima, Japan

[8] Fukushima Med Univ, Adv Clin Res Ctr, Fukushima Global Med Sci Ctr, Fukushima, Japan

[9] Ehime Prefectural Cent Hosp, Dept Neurol, Matsuyama, Ehime, Japan

[10] Keiyu Hosp, Dept Neurol, Yokohama, Kanagawa, Japan

来源：

NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION | 2017年 / 4卷 / 06期

关键词：

REFRACTORY STATUS EPILEPTICUS; ASPARTATE RECEPTOR ENCEPHALITIS; MEDIATED STATUS EPILEPTICUS; ACUTE ENCEPHALOPATHY; ANTIBODIES; EPILEPSY; ADULTS; CHILDHOOD; SEIZURES; OUTCOMES;

D O I：

10.1212/NXI.0000000000000396

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Objective: 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. Methods: 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). Results: 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. Conclusions: 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.

引用

页数：9

共 39 条

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