Autoantibodies to glutamate receptor GluRepsilon2 in a patient with limbic encephalitis associated with relapsing polychondritis.
Limbic encephalitis is a rare central nervous system (CNS) manifestation of relapsing polychondritis (RP). Vasculitis is assumed to be the cause of CNS involvement in RP. Several studies, however, have described CNS involvement in RP with no evidence of vasculitis but with a more nonspecific inflammatory picture. We report a patient with limbic encephalitis associated with RP who presented with anti-glutamate receptor (GluR) epsilon2 (NR2B) autoantibodies in his cerebrospinal fluid and sera. Brain MRI showed a high signal intensity lesion in the medial temporal lobe and progressive atrophy without multifocal abnormality on fluid-attenuated inversion recovery scanning. Our patient's results raise the interesting possibility that anti-GluRepsilon2 (NR2B) antibodies function in the development of limbic encephalitis in certain patients with RP.
Kashihara
Kenichi
K
Department of Neurology, Okayama Kyokuto Hospital, Okayama 703-8265, Japan. kkashi@kyokuto.or.jp
Kawada
Sanami
S
Takahashi
Yukitoshi
Y
D000679:Amygdala / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D001284:Atrophy / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D001323:Autoantibodies / Q000032:analysis / Q000097:blood / Q000134:cerebrospinal fluid; D015415:Biomarkers; D001921:Brain / Q000276:immunology* / Q000473:pathology* / Q000503:physiopathology; D002356:Cartilage / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D018450:Disease Progression; D018698:Glutamic Acid / Q000378:metabolism; D006624:Hippocampus / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D020363:Limbic Encephalitis / Q000276:immunology* / Q000473:pathology* / Q000503:physiopathology; D008032:Limbic System / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D009410:Nerve Degeneration / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D010672:Phenytoin / Q000627:therapeutic use; D011081:Polychondritis, Relapsing / Q000150:complications*; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D012640:Seizures / Q000209:etiology; D009435:Synaptic Transmission / Q000276:immunology; D013702:Temporal Lobe / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D000927:Anticonvulsants; D001323:Autoantibodies; D015415:Biomarkers; C121001:NR2B NMDA receptor; D016194:Receptors, N-Methyl-D-Aspartate; D018698:Glutamic Acid; D010672:Phenytoin; D008775:Methylprednisolone