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

Jung Won Shin, Yong Seo Koo, Young Soo Kim, Dong Wook Kim, Kwang Ki Kim, Seo Young Lee, Hyun Kyung Kim, Hye Jin Moon, Jung Ah Lim, Jung Ick Byun, Jun Sang Sunwoo, Jangsup Moon, Soon Tae Lee, Keun Hwa Jung, Kyung Il Park, Kon Chu, Jae Moon Kim, Yong Won Cho, Ki Young Jung, Sang Kun Lee

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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 3–6 months 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 3–6 months 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.

Original languageEnglish
Pages (from-to)1-8
Number of pages8
JournalJournal of Neuroimmunology
Volume315
DOIs
StatePublished - 15 Feb 2018

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Status Epilepticus
Encephalitis
Multicenter Studies
Cohort Studies
Immunotherapy
Active Immunotherapy
Patient Discharge
Autoantibodies
Registries
Antibodies

Keywords

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

Cite this

Shin, Jung Won ; Koo, Yong Seo ; Kim, Young Soo ; Kim, Dong Wook ; Kim, Kwang Ki ; Lee, Seo Young ; Kim, Hyun Kyung ; Moon, Hye Jin ; Lim, Jung Ah ; Byun, Jung Ick ; Sunwoo, Jun Sang ; Moon, Jangsup ; Lee, Soon Tae ; Jung, Keun Hwa ; Park, Kyung Il ; Chu, Kon ; Kim, Jae Moon ; Cho, Yong Won ; Jung, Ki Young ; Lee, Sang Kun. / Clinical characterization of unknown/cryptogenic status epilepticus suspected as encephalitis : A multicenter cohort study. In: Journal of Neuroimmunology. 2018 ; Vol. 315. pp. 1-8.
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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 3–6 months 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 3–6 months 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.",
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Clinical characterization of unknown/cryptogenic status epilepticus suspected as encephalitis : A multicenter cohort study. / Shin, Jung Won; Koo, Yong Seo; Kim, Young Soo; Kim, Dong Wook; Kim, Kwang Ki; Lee, Seo Young; Kim, Hyun Kyung; Moon, Hye Jin; Lim, Jung Ah; Byun, Jung Ick; Sunwoo, Jun Sang; Moon, Jangsup; Lee, Soon Tae; Jung, Keun Hwa; Park, Kyung Il; Chu, Kon; Kim, Jae Moon; Cho, Yong Won; Jung, Ki Young; Lee, Sang Kun.

In: Journal of Neuroimmunology, Vol. 315, 15.02.2018, p. 1-8.

Research output: Contribution to journalArticle

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T1 - Clinical characterization of unknown/cryptogenic status epilepticus suspected as encephalitis

T2 - A multicenter cohort study

AU - Shin, Jung Won

AU - Koo, Yong Seo

AU - Kim, Young Soo

AU - Kim, Dong Wook

AU - Kim, Kwang Ki

AU - Lee, Seo Young

AU - Kim, Hyun Kyung

AU - Moon, Hye Jin

AU - Lim, Jung Ah

AU - Byun, Jung Ick

AU - Sunwoo, Jun Sang

AU - Moon, Jangsup

AU - Lee, Soon Tae

AU - Jung, Keun Hwa

AU - Park, Kyung Il

AU - Chu, Kon

AU - Kim, Jae Moon

AU - Cho, Yong Won

AU - Jung, Ki Young

AU - Lee, Sang Kun

PY - 2018/2/15

Y1 - 2018/2/15

N2 - 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 3–6 months 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 3–6 months 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.

AB - 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 3–6 months 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 3–6 months 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.

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