Rinsho Shinkeigaku (Clinical Neurology)

The 49th Annual Meeting of the Japanese Society of Neurology

Relationship between anti-NMDAR encephalitis and acute juvenile female non-herpetic encephalitis (AJFNHE)

Satoshi Kamei, M.D.

Division of Neurology, Department of Medicine, Nihon University School of Medicine

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)

(CLINICA NEUROL, 48: 916|919, 2008)
key words: acute juvenile female non-herpetic encephalitis, anti-N-methyl-d-aspartate receptor encephalitis, nationwide survey, ovarian teratoma

(Received: 16-May-08)