ABSTRACT
We compared clinical characteristics and autoantibodies against GluRepsilon2 between 95 patients with nonparaneoplastic 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 GluRepsilon2 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 GluRepsilon2. Antibodies against NMDAR complex (Dalmau's method) in CSF were detected in 90.9% (10/11) of NHAE-OT patients.
Subject(s)
Limbic Encephalitis , Receptors, N-Methyl-D-Aspartate/immunology , Acute Disease , Adolescent , Adult , Aged , Aged, 80 and over , Autoantibodies , Child , Child, Preschool , Epitopes , Female , Humans , Male , Middle Aged , Ovarian Neoplasms , Teratoma , Young AdultABSTRACT
We examined autoantibodies against GluRepsilon2 in patients with acute encephalitis, who were categorized into localized encephalitis and widespread encephalitis. Patients with localized encephalitis are defined as patients showing psychic symptoms (illusions, anxiety and distraction etc.), solitary seizures and/or very mild impairment of consciousness in the initial stage. Patients with widespread encephalitis are defined as patients showing a profound loss of consciousness and or convulsive status in the initial stage. In 24 patients with localized encephalitis, immunoglobulin (Ig) M autoantibodies against GluRepsilon2 tended to appear in CSF in the acute stage (0-20 days after onset of neurological symptoms) or recovery stage (21-60 days after onset of neurological symptoms) of encephalitis. In 22 patients with widespread encephalitis, IgM autoantibodies against GluRepsilon2 in CSF tended to appear in the recovery stage (21-60 days after onset of neurological symptoms) or chronic stage (>60 days after onset of neurological symptoms) of encephalitis. All patients with localized encephalitis had autoantibodies to the extracellular N epitope. However, no patients with widespread encephalitis had autoantibodies to the extracellular N epitope in acute stages. These data may suggest that GluR autoimmunity contributes to the onset of localized encephalitis.
