Elucidation of clinical characteristics, brain images and immunological pathophysiology in childhood NMDAR encephalitis

2023 Fiscal Year Final Research Report

• Project/Area Number: 21K07788
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 52050:Embryonic medicine and pediatrics-related
• Research Institution: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders
• Principal Investigator: Takahashi Yukitoshi 独立行政法人国立病院機構(静岡・てんかん神経医療センター臨床研究部), その他部局等, その他 (70262764)
• Project Period (FY): 2021-04-01 – 2024-03-31
• Keywords: 抗NMDA受容体脳炎 / 自己抗体 / グルタミン酸受容体 / 痙攣重積 / 重症薬疹 / ショック

Outline of Final Research Achievements

In our study of 38 definite cases of childhood-onset anti-NMDA receptor encephalitis diagnosed according to Dalmau et al.'s diagnostic criteria for anti-NMDA receptor encephalitis (2019), Not every case could be diagnosed as probable anti-NMDA receptor encephalitis, based only on characteristic laboratory findings without autoantibodies or characteristic symptoms. The measurement of anti-NMDAR antibodies in CSF was considered essential for early diagnosis.
In anti-NMDA receptor encephalitis (136 definite cases, including adults), we examined acute clinical factors affecting prognosis, and found that the appearance of acute symptomatic seizures, persistence of status epilepticus, and severe treatment complications (cardiopulmonary circulatory complications such as sinus arrest and shock, and severe infections such as sepsis) significantly affected prognosis.

Free Research Field

小児神経

Academic Significance and Societal Importance of the Research Achievements

抗NMDA受容体脳炎の早期診断は予後改善に不可欠であるが、Dalmauらの診断基準にある特徴的臨床症状は確定症例の60.9％の症例で揃い、髄液細胞増多は74.3％が満たしていたにすぎず、臨床症状と髄液細胞増多のみでは、抗NMDAR脳炎を見落とすことになることが分かった。よって、早期診断のためにはNMDAR抗体（CBA）を早期に測定し、早期に結果が臨床医に報告される必要がある。
抗NMDA受容体脳炎の予後に大きく影響する、重症治療合併症（洞停止、ショックなどの心肺循環系合併症、敗血症など）を防止することが重要で、人工呼吸器、持続鎮静、血漿交換治療などにおいては、集中的な監視と早期対応が望まれる。