中枢神経系炎症性脱ミエリン症の小児患者の急性症状発作および
Shangru Li1, Xuting Chang1, Jie Zhang1
1Children's Medical Center, Peking University First Hospital, Beijing 102627, China.
Multiple sclerosis and related disorders
|August 31, 2025
まとめ
急性症状性発作は,中枢神経系炎症性脱ミエリン性疾患 (CIDD) を患っている子供の3分の1以上,特にMOGADまたは血清陰性CIDDを患っている子供に影響します. これらの子供の9.9%で発症します.
科学分野:
- 小児神経学
- 神経免疫学
- 脱エリン化 する 病気
背景:
- 中枢神経系炎症性脱ミエリン性疾患 (CIDD) は,脳と脊髄に影響する獲得免疫媒介疾患のグループを網羅する.
- 急性症状性発作 (Acute symptomatic seizures (ASS)) とは,小児性CIDDにおける潜在的な神経学的合併症であり,患者のアウトカムに影響を与える.
- 発作の流行病学とリスク要因を理解することは 早期の診断と管理に不可欠です
研究 の 目的:
- 中枢神経系炎症性脱ミエリン性疾患 (CIDDs) を診断した子供における急性症状性発作 (ASSs) との罹患率を決定する.
- この小児科コホートにおけるASSの発生との発症に関連する臨床的および放射線学的リスク要因を特定する.
- CIDD の異なるサブタイプにおける ASS の発生率を比較する.
主な方法:
- 2013年1月から2024年6月まで,北京大学第一病院でCIDDと診断された子供たちに遡及的なコホート研究が行われました.
- 様々なCIDD分類の患者の間でASSの発生率を評価し,比較した.
- 発作の特徴,脳MRIの発見,疾患の進行を含む臨床データは,ASSとの危険因子を特定するために分析されました.
主要な成果:
- CIDDを患った204人のうち75人 (36. 8%) がASSを経験しました. 発症率はミエリンオリゴデンドロサイト抗体関連疾患 (MOGAD) と血清陰性CIDDで高かった.
- ASSは,急性分散脳炎 (ADEM) と脳皮質脳炎 (CCE) のフェノタイプとして発現する発作の頻度が高い.
- エピレプシーの全般的な罹患率は9. 9%で,多発性脱ミエリン発作は発症の予測因子である. 発熱とMRIにおける皮質の関与は,ASSに対する独立した保護因子であった.
結論:
- CIDDを持つ子供の有意な割合はASSを経験し,MOGADとセロネガティブなCIDDはより高い発生率を示しています.
- ADEMとCCEのフェノタイプのような特定の臨床表現は,ASSのリスクの増加と関連しています.
- は顕著な長期的な合併症で,CIDDの子供のほぼ10%に影響を与え,特に再発性脱ミエリン症の子供です.
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