Reversible Brain Atrophy in Cryptogenic New-onset Refractory Status Epilepticus

Tomoaki Watanabe; Masahiro Nakamori; Kenichi Ishikawa; Atsuko Motoda; Shuichiro Neshige; Shotaro Yamamoto; Kento Katahira; Toru Higaki; Hiroki Ueno; Takahiro Iizuka; Hirofumi Maruyama

doi:10.2169/internalmedicine.0465-22

CASE REPORTS

Reversible Brain Atrophy in Cryptogenic New-onset Refractory Status Epilepticus

Tomoaki Watanabe, Masahiro Nakamori, Kenichi Ishikawa, Atsuko Motoda, Shuichiro Neshige, Shotaro Yamamoto, Kento Katahira, Toru Higaki, Hiroki Ueno, Takahiro Iizuka, Hirofumi Maruyama

Author information

Tomoaki Watanabe
Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Japan
Masahiro Nakamori
Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Japan
Kenichi Ishikawa
Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Japan
Atsuko Motoda
Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Japan
Shuichiro Neshige
Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Japan
Shotaro Yamamoto
Department of Internal Medicine, Mitsugi General Hospital, Japan
Kento Katahira
Department of Rehabilitation, Mitsugi General Hospital, Japan
Toru Higaki
Graduate School of Advanced Science and Engineering, Hiroshima University, Japan
Hiroki Ueno
Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Japan
Takahiro Iizuka
Department of Neurology, Kitasato University School of Medicine, Japan
Hirofumi Maruyama
Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Japan

Keywords: cryptogenic new-onset refractory status epilepticus, intensive immunotherapy, reversible brain atrophy, MRI, autoantibodies

JOURNAL OPEN ACCESS

2023 Volume 62 Issue 12 Pages 1835-1842

DOI https://doi.org/10.2169/internalmedicine.0465-22

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Abstract

Cryptogenic new-onset refractory status epilepticus (C-NORSE) is a neurologic emergency condition characterized by refractory status epilepticus (RSE) of unknown cause. Brain atrophy in a setting of C-NORSE is usually irreversible. A 33-year-old woman who was highly suspected of C-NORSE once showed mild frontotemporal atrophy on brain magnetic resonance imaging (MRI), but follow-up MRI revealed recovery of the brain atrophy. Her cognitive function also gradually improved, with a reduction in seizure frequency. Early initiation of intensive immunotherapy with anti-seizure medications may have minimized irreversible brain damage associated with RSE, resulting in a relatively good outcome.

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