title: Autoimmune channelopathies: well-established and emerging immunotherapy-responsive diseases of the peripheral and central nervous systems.
abstract: BACKGROUND
  The role of antibodies in neuromuscular junction disorders is well established with antibodies to acetylcholine receptor, muscle-specific kinase, and voltage-gated calcium channels. The diseases associated with these antibodies, myasthenia gravis and the Lambert-Eaton myasthenic syndrome, respond well to symptomatic treatments (e.g., cholinesterase inhibitors) and to immunotherapies such as plasma exchange, intravenous immunoglobulin, oral steroids, and steroid-sparing drugs. The role of the antibodies has been established by a variety of in vitro and in vivo approaches. More recently, antibodies to voltage-gated potassium channels have been identified in patients with autoimmune forms of acquired neuromyotonia. Over the last decade, antibodies to CNS membrane receptors or ion channels have begun to be identified and these antibodies define antibody-mediated CNS diseases that also respond to immunotherapies.
  
  
  CONCLUSIONS
  The paradigms gained from the study of the peripheral conditions has led to a better appreciation of the role of antibodies in neurological disorders and a growing recognition of their role in central nervous system (CNS) diseases.
authors.affiliation: Department of Clinical Neurology, Level 6 West Wing, John Radcliffe Hospital, University of Oxford, Oxford, UK. angela.vincent@imm.ox.ac.uk
authors.forename: Angela
authors.initials: A
authors.lastname: Vincent
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D020274:Autoimmune Diseases of the Nervous System / Q000276:immunology / Q000628:therapy; D015220:Calcium Channels / Q000276:immunology; D053447:Channelopathies / Q000276:immunology / Q000628:therapy; D005968:Glutamate Decarboxylase / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007167:Immunotherapy; D007231:Infant, Newborn; D020294:Myasthenic Syndromes, Congenital / Q000276:immunology / Q000628:therapy; D020361:Paraneoplastic Syndromes, Nervous System / Q000276:immunology / Q000628:therapy; D010951:Plasma Exchange; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology; D017981:Receptors, Neurotransmitter / Q000276:immunology
chemical_list: C496030:AQP4 protein, human; D000305:Adrenal Cortex Hormones; D051401:Aquaporin 4; D001323:Autoantibodies; D001324:Autoantigens; D015220:Calcium Channels; D016756:Immunoglobulins, Intravenous; D024642:Potassium Channels, Voltage-Gated; D017981:Receptors, Neurotransmitter; D005968:Glutamate Decarboxylase
references.citation: Neurology. 1996 Sep;47(3):678-83
references.pmid: 8797464
references.citation: Nat Med. 2001 Mar;7(3):365-8
references.pmid: 11231638
references.citation: Ann Neurol. 1997 Feb;41(2):238-46
references.pmid: 9029073
references.citation: Brain. 2008 Nov;131(Pt 11):3072-80
references.pmid: 18945724
references.citation: Brain. 2008 Jul;131(Pt 7):1940-52
references.pmid: 18515870
references.citation: Neurology. 2005 Dec 13;65(11):1730-6
references.pmid: 16344514
references.citation: J Neurol Neurosurg Psychiatry. 2006 May;77(5):704-5
references.pmid: 16614042
references.citation: Muscle Nerve. 1998 May;21(5):672-4
references.pmid: 9572254
references.citation: Neuron. 2006 Oct 5;52(1):123-38
references.pmid: 17015231
references.citation: Ann N Y Acad Sci. 2008;1132:163-73
references.pmid: 18567866
references.citation: Neurology. 2004 Apr 13;62(7):1177-82
references.pmid: 15079019
references.citation: Teratology. 2000 Nov;62(5):332-41
references.pmid: 11029151
references.citation: Neurology. 2008 Nov 11;71(20):1647-8
references.pmid: 18815385
references.citation: Ann N Y Acad Sci. 2003 Sep;998:187-95
references.pmid: 14592874
references.citation: Ann Neurol. 2009 Nov;66(5):630-43
references.pmid: 19937948
references.citation: Brain. 2002 Aug;125(Pt 8):1887-95
references.pmid: 12135978
references.citation: Nat Rev Immunol. 2002 Oct;2(10):797-804
references.pmid: 12360217
references.citation: Ann Neurol. 2009 Nov;66(5):704-9
references.pmid: 19938173
references.citation: Ann Neurol. 2009 Nov;66(5):617-29
references.pmid: 19938104
references.citation: Lancet Neurol. 2008 Apr;7(4):327-40
references.pmid: 18339348
references.citation: Ann Neurol. 2009 Jul;66(1):11-8
references.pmid: 19670433
references.citation: Lancet. 1991 Jul 13;338(8759):75-7
references.pmid: 1676468
references.citation: Brain. 2010 Feb;133(Pt 2):349-61
references.pmid: 20047900
references.citation: Lancet. 1995 Jul 1;346(8966):24-5
references.pmid: 7603140
references.citation: Ann Neurol. 2003 Apr;53(4):533-7
references.pmid: 12666123
references.citation: Biochem Biophys Res Commun. 2009 Sep 4;386(4):623-7
references.pmid: 19545538
references.citation: J Exp Med. 2008 Oct 27;205(11):2473-81
references.pmid: 18838545
references.citation: J Clin Neurophysiol. 2004 Nov-Dec;21(6):440-5
references.pmid: 15622131
references.citation: Ann Neurol. 2007 Jan;61(1):25-36
references.pmid: 17262855
references.citation: Science. 1975 Oct 24;190(4212):397-9
references.pmid: 1179220
references.citation: Neurology. 1978 Mar;28(3):266-72
references.pmid: 564482
references.citation: N Engl J Med. 1977 Jan 20;296(3):125-31
references.pmid: 831074
references.citation: J Clin Invest. 1999 Apr;103(7):1031-8
references.pmid: 10194476
references.citation: Nat Clin Pract Neurol. 2008 Apr;4(4):202-14
references.pmid: 18334978
references.citation: Lancet. 2004 Dec 11-17;364(9451):2106-12
references.pmid: 15589308
references.citation: Ann Neurol. 1995 Nov;38(5):714-22
references.pmid: 7486862
references.citation: Brain. 2004 Mar;127(Pt 3):701-12
references.pmid: 14960497
references.citation: Brain. 2001 Dec;124(Pt 12):2417-26
references.pmid: 11701596
references.citation: Neurology. 2008 Dec 9;71(24):2008-10
references.pmid: 19064883
references.citation: Neurology. 2008 Oct 14;71(16):1291-2
references.pmid: 18852446
references.citation: Ann Neurol. 2001 Jul;50(1):73-8
references.pmid: 11456313
references.citation: Neurology. 2007 Dec 11;69(24):2221-31
references.pmid: 17928579
references.citation: J Exp Med. 2005 Aug 15;202(4):473-7
references.pmid: 16087714
references.citation: Epilepsy Res. 2006 Oct;71(2-3):135-41
references.pmid: 16870397
references.citation: Arch Neurol. 2008 Jul;65(7):913-9
references.pmid: 18625857
references.citation: Muscle Nerve Suppl. 2002;11:S55-8
references.pmid: 12116286