Rinsho Shinkeigaku (Clinical Neurology)

The 49th Annual Meeting of the Japanese Society of Neurology

Acute limbic encephalitis and NMDA type-glutamate receptor

Yukitoshi Takahashi, M.D.1)2), Etsuko Yamazaki, M.D.1), Shigeko Nishimura, M.D.1), Hisano Tsunogae, M.D.1), Kenji Niwa, M.D.3), Josep Dalmau, M.D.4), Katsumi Imai, M.D.1) and Tateki Fujiwara, M.D.1)

1)National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders
2)Department of Pediatrics, Gifu University School of Medicine
3)Department of Obstetricus, Gifu University School of Medicine
4)Department of Neurology, University of Pennsylvania

We compared clinical characteristics and autoantibodies against GluRε2 between 95 patients with non-paraneoplastic non-herpetic acute limbic encephalitis (NPNHALE) and 19 patients with non-herpetic acute encephalitis accompanying ovarian teratoma (NHAE-OT).
Onset age (mean±SD) was 27.7±18.6 years old in NPNHALE, 27.5±6.5 in NHALE-OT. Preceding factors were found in 63.8% of patients with NPNHALE and 89.5% of patients with NHALE-OT (Fisher's exact test, p=0.025), and major preceding factors were upper respiratory infections or fever in both groups. Symptoms at the onset were disorder of behavior and talk > seizures > impairment of consciousness in NPNHALE, and disorder of behavior and talk > seizures > disorientation in NHALE-OT. Symptoms at the acute stage were similar between NPNHALE and NHAE-OT, but duration of hospital stay was longer in NHAE-OT (209.0 days) than NPNHALE (87.5 days)(Mann Whitney test, p<0.0001). At the onset, cell counts in CSF were 51.6±66.4/mm3 and protein levels were 35.4±14.7 mg/dl, and IgG levels were 6.6±4.2 mg/dl in NHAE-OT, and these data were not significantly different between NPNHALE and NHAE-OT.
In acute stage, autoantibodies against whole molecule of GluRε2 in CSF were detected in 51.8% (29/56) of adult NPNHALE, and 40% (6/15) of NHAE-OT patients by immunoblot. These autoantibodies in both groups included epitopes to n-terminal of GluRε2. Antibodies against NMDAR complex (Dalmau's method) in CSF were detected in 90.9% (10/11) of NHAE-OT patients.
Full Text of this Article in Japanese PDF (358K)

(CLINICA NEUROL, 48: 926－929, 2008)
key words: acute limbic encephalitis, NMDA type glutamate receptor, autoantibodies, ovarian teratoma

(Received: 16-May-08)