New autoantibody mediated disorders of the central nervous system.
OBJECTIVE
Recently, central nervous system disorders have been shown to be associated with autoantibodies. This review summarizes the recent findings and assesses the evidence that these conditions are caused by the antibodies, using the criteria established for peripheral nervous system autoimmune diseases.


RESULTS
Over the last few years, antibodies to voltage-gated calcium and potassium channels, and to glutamate receptors, have been detected in the serum and cerebrospinal fluid of patients with ataxia, limbic encephalitis and certain forms of epilepsy. Some of these patients respond to immunotherapies, suggesting that the antibodies are pathogenic, but there are few demonstrations using the passive transfer approach that antibodies present in the serum can penetrate the blood-brain barrier and affect central nervous system function. Some patients have antibodies to intracellular proteins such as glutamic acid decarboxylase or specific ribonuclear proteins. The pathogenicity of these antibodies must be in some doubt, although intravenous immunoglobulin therapy has been shown to be beneficial in stiff man syndrome, consistent with an autoimmune aetiology for the disease. In only a few conditions, has IgG derived from patients been shown to produce pathogenic effects in vivo or in vitro.


CONCLUSIONS
There is much that needs to be done to define the role of these antibodies and to determine how they affect central nervous system function in vivo. These studies must be carried out so that appropriate treatments can be provided for the growing number of patients with possible antibody-mediated conditions.
Lang
Bethan
B
Neurosciences Group, Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, University of Oxford, UK. blang@hammer.imm.ox.ac.uk
Dale
Russell C
RC
Vincent
Angela
A
D000818:Animals; D001323:Autoantibodies / Q000097:blood / Q000134:cerebrospinal fluid; D001324:Autoantigens; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology; D020274:Autoimmune Diseases of the Nervous System / Q000276:immunology*; D015220:Calcium Channels / Q000276:immunology; D002446:Celiac Disease / Q000276:immunology; D002493:Central Nervous System Diseases / Q000276:immunology*; D002524:Cerebellar Ataxia / Q000276:immunology; D004660:Encephalitis / Q000276:immunology; D004827:Epilepsy / Q000276:immunology; D005968:Glutamate Decarboxylase / Q000276:immunology; D015368:Human T-lymphotropic virus 1 / Q000276:immunology; D006801:Humans; D020363:Limbic Encephalitis / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000276:immunology; D020362:Paraneoplastic Cerebellar Degeneration / Q000276:immunology; D015221:Potassium Channels / Q000276:immunology; D018091:Receptors, AMPA; D017470:Receptors, Glutamate / Q000276:immunology; D013290:Streptococcal Infections / Q000150:complications / Q000276:immunology; D013595:Syringomyelia / Q000276:immunology
D001323:Autoantibodies; D001324:Autoantigens; D015220:Calcium Channels; D015221:Potassium Channels; D018091:Receptors, AMPA; D017470:Receptors, Glutamate; C104723:glutamate receptor ionotropic, AMPA 3; D005968:Glutamate Decarboxylase