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J Neuroimmunol. 2018 Feb 15;315:1-8. doi: 10.1016/j.jneuroim.2017.12.004. Epub 2017 Dec 12.

Clinical characterization of unknown/cryptogenic status epilepticus suspected as encephalitis: A multicenter cohort study.

Author information

1
Department of Neurology, CHA University, Bundang CHA Medical Center, Republic of Korea.
2
Department of Neurology, Korea University Anam Hospital, Korea University Medicine Center, Republic of Korea; Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Republic of Korea.
3
Department of Neurology, Gyeongsang National University Hospital, Republic of Korea.
4
Department of Neurology, Konkuk University School of Medicine, Republic of Korea.
5
Department of Neurology, Dongguk University Ilsan Hospital, Republic of Korea.
6
Department of Neurology, Kangwon National University Hospital, Republic of Korea.
7
Department of Neurology, National Medical Center, Republic of Korea.
8
Department of Neurology, Soonchunhyang University Bucheon Hospital, Republic of Korea.
9
Department of Neurology, National Center for Mental Health, An Affiliate of the Ministry for Health & Welfare, Republic of Korea.
10
Department of Neurology, Kyung Hee University Hospital, Gangdong, Republic of Korea.
11
Department of Neurology, Soonchunhyang University Seoul Hospital, Republic of Korea.
12
Department of Neurology, Seoul National University Hospital, Republic of Korea.
13
Department of Neurology, Seoul National University Hospital, Republic of Korea; Department of Neurology, Seoul National University Hospital Healthcare System Gangnam Center, Republic of Korea.
14
Department of Neurology, Chungnam National University Hospital, Republic of Korea.
15
Department of Neurology, Dongsan Medical Center, Keimyung University, Republic of Korea.
16
Department of Neurology, Seoul National University Hospital, Republic of Korea. Electronic address: jungky@snu.ac.kr.
17
Department of Neurology, Seoul National University Hospital, Republic of Korea. Electronic address: sangkun@snu.ac.kr.

Abstract

Autoimmune and unknown/cryptogenic encephalitis have been increasingly noted in the inflammatory etiology of new-onset status epilepticus (SE). We aimed to investigate clinical characteristics and the potential role of immunotherapy in encephalitis-related adult SE through our multicenter prospective SE registry. Among the 274 patients with SE, 35 (12.8%) patients demonstrated an inflammatory etiology and 19 out of 35 (54.3%) patients demonstrated unknown/cryptogenic cause. Patients with autoimmune and unknown/cryptogenic encephalitis shared similar clinical features. In unknown/cryptogenic encephalitis, the proportion of favorable outcomes (mRS 0-3) showed a different propensity at 3-6months after discharge between patients receiving active immunotherapy and not receiving any immunotherapy, although it was not statistically significant (at admission 28.6% vs 20%, p=0.603; at discharge 57.1% vs 60%, p=0.570; at 3-6months after discharge 90% vs 60%, p=0.214 in patients treated with active immunotherapy or without immunotherapy, respectively). Extensive autoantibody screening should be carried out and empirical immunotherapy may be potentially helpful even in patients without antibodies, although longer term and multi-national studies may be necessary to make a stronger recommendation.

KEYWORDS:

Autoimmune encephalitis; Immunotherapy; Inflammatory CNS disease; Status epilepticus; Unknown/cryptogenic

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