臨床神経学

第49回日本神経学会総会

<シンポジウム3-1>抗NMDA受容体抗体陽性脳症
若年女性に好発する急性非ヘルペス性脳炎(AJFNHE)との関係・異同

亀井 聡

日本大学医学部内科学系神経内科学分野〔〒173-8610 東京都板橋区大谷口上町30-1〕

The relationships between AJFNHE (Kamei S: 2004) and anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis (Dalmau J et al: 2007) is discussed. The comparative clinical features in both reports revealed that most were young adult women, a prodrome was presented in 80%, the first neurological symptom was psychosis, the main symptoms included convulsions, consciousness disturbance, and involuntary movements, and mechanical ventilation was required in 80%. Dalmau reported that ovarian teratoma was demonstrated in 11 out of 12 patients, and mediastinal teratoma in 1 patient. We had yet to examine these conditions at the time of publication. AJFNHE and anti-NMDAR encephalitis are thus considered to be the same condition. AJFNHE represents a clinical concept based on the specific clinical features, and anti-NMDAR encephalitis represents a clinical entity based on the neuro-oncological findings. A nationwide survey of AJFNHE was undertaken in Japan. Collected patients predominantly were young adult women. Their clinical features were uniform and also in concordance with those previously reported as AJFNHE. This survey revealed that the annual incidence was 0.33/106 population, respiratory failure was observed in 70% and required care with mechanical ventilation, associated tumors were demonstrated in 40% and ovarian teratoma was the most frequent.
Full Text of this Article in Japanese PDF (293K)

(臨床神経, 48:916−919, 2008)
key words:若年女性に好発する急性非ヘルペス性脳炎, 抗NMDAR脳炎, 全国調査, 卵巣奇形腫

(受付日:2008年5月16日)