Contribution of activated complements and CD59 to the pathophysiology of Rasmussen syndrome

Rasmussen syndrome (RS) is an autoimmune-associated epilepsy with refractory seizures and deteriorating motor and cognitive functions. We expect to unveil complex immunological pathophysiology from the viewpoint of complements to establish newer immunomodulatory treatments. We examined CSF levels of activated complements (iC3b, C5a and soluble membrane attack complex (sMAC)) and CD59 (inhibitor of MAC) using ELISA kits in 36 patients with RS and 44 disease controls (DCs). Disease duration from the onset of RS to CSF examination (mean ± SD) was 80.5 ± 89.7 months (n = 73). Seizure frequency at CSF examination was daily in 56, weekly in four, monthly in six, yearly in four and seizure-free in three CSF samples. The level of iC3b was significantly higher in RS than in DCs, but that of C5a, sMAC or CD59 was not different between RS and DCs. Levels of iC3b increased along with duration of disease. The ratio of patients with the levels of CD59 more than zero increased after the acute stage. Levels of CD59 in patients with daily seizure frequency were significantly lower than those with seizure control. The odds ratio between levels of CD59 and IVIg treatment by multiple linear regression analyses was significantly high. Maximum datum of activated complements or CD59 among the course of each patient was not related with final motor, cognitive and seizure outcomes. Reduction of CD59 may be one of crucial pathologies in the early stage of RS.

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Journal
Journal of Neuroimmunology
Published
2025-06-13
DOI
https://doi.org/10.1016/j.jneuroim.2025.578667
Citations
1
Primary Topic
Cystic Fibrosis Research Advances
Type
article
Field-Weighted Citation Impact
1.52

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article

Contribution of activated complements and CD59 to the pathophysiology of Rasmussen syndrome

Tokito Yamaguchi, Chihiro Yonee, Shinobu Fukumura, Masataka Fukuoka et al.
1 citations
Journal of Neuroimmunology
Cystic Fibrosis Research Advances
1.52
article

Contribution of activated complements and CD59 to the pathophysiology of Rasmussen syndrome

Tokito Yamaguchi, Chihiro Yonee, Shinobu Fukumura, Masataka Fukuoka, Emiko Takao, Yukiko Mogami, Shigeko Nishimura, Hirofumi Kashii, Kenichi MAEDA, Youhei Himeno, Osamu Kawano, Naoki Hayashi, Yoshinori Okumura, Risa Kasai, Natsumi Saito, Yasukiyo Araki, Keisuke Watanabe, Takefumi Higashijima, Yasuko Kuroha, Misa Nakamura, Yukitoshi Takahashi, Yosuke Miyamoto, Yoshiyuki Kondo, Kazunori Ogawa, Tamami Yano, Minoru Abe, Osamu Kobayashi
article en
1 citations

Abstract

Rasmussen syndrome (RS) is an autoimmune-associated epilepsy with refractory seizures and deteriorating motor and cognitive functions. We expect to unveil complex immunological pathophysiology from the viewpoint of complements to establish newer immunomodulatory treatments. We examined CSF levels of activated complements (iC3b, C5a and soluble membrane attack complex (sMAC)) and CD59 (inhibitor of MAC) using ELISA kits in 36 patients with RS and 44 disease controls (DCs). Disease duration from the onset of RS to CSF examination (mean ± SD) was 80.5 ± 89.7 months (n = 73). Seizure frequency at CSF examination was daily in 56, weekly in four, monthly in six, yearly in four and seizure-free in three CSF samples. The level of iC3b was significantly higher in RS than in DCs, but that of C5a, sMAC or CD59 was not different between RS and DCs. Levels of iC3b increased along with duration of disease. The ratio of patients with the levels of CD59 more than zero increased after the acute stage. Levels of CD59 in patients with daily seizure frequency were significantly lower than those with seizure control. The odds ratio between levels of CD59 and IVIg treatment by multiple linear regression analyses was significantly high. Maximum datum of activated complements or CD59 among the course of each patient was not related with final motor, cognitive and seizure outcomes. Reduction of CD59 may be one of crucial pathologies in the early stage of RS.

Journal of NeuroimmunologyVol. 406
University of Miyazaki (JP), Sapporo Medical University (JP), Oita University (JP), Kyorin University (JP), Akita University (JP), Kyoto Prefectural University of Medicine (JP), National Epilepsy Center (JP), Osaka Women's and Children's Hospital (JP), Kurume University Hospital (JP), Kariya Toyota General Hospital (JP), National Center of Neurology and Psychiatry (JP), Hiroshima City Asa Citizens Hospital (JP), Tokyo Metropolitan Neurological Hospital (JP), Nishi Niigata Chuo National Hospital (JP), Shizuoka Children's Hospital (JP)
Japan Intractable Diseases Research Foundation, Ministry of Health, Labour and Welfare, Japan Epilepsy Research Foundation
Zero hunger
Openalex Percentile: Top 17%
Cystic Fibrosis Research Advances
1.52
DOIOpen Access PDFOpenAlex
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