title: Are onconeural antibodies a clinical phenomenology in paraneoplastic limbic encephalitis?
abstract: Paraneoplastic neurological syndromes (PNSs) occur in patients with cancer and can cause clinical symptoms and signs of dysfunction of the nervous system that are not due to a local effect of the tumor or its metastases. Most of these clinical syndromes in adults are associated with lung cancer, especially small cell lung cancer (SCLC), lymphoma, and gynecological tumors. The finding of highly specific antibodies directed against onconeural antigens has revolutionized the diagnosis and promoted the understanding of these syndromes and led to the current hypothesis of an autoimmune pathophysiology. Accumulating data strongly suggested direct pathogenicity of these antibodies. The field of PNS has expanded rapidly in the past few years with the discovery of limbic encephalitis associated with glutamic acid decarboxylase (GAD) 65, the voltage (VGKC-gated potassium channel) complex, the methyl (N-NMDA-D-aspartate), alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA), and gamma aminobutyric acid (GABA) (B) receptors, and so forth. Despite this, the clinical spectrum of these diseases has not yet been fully investigated. The clinical importance of these conditions lies in their frequent response to immunotherapies and, less commonly, their association with distinctive tumors. This review provides an overview on the pathogenesis and diagnosis of PNS, with emphasis on the role of antibodies in limbic encephalitis.
authors: Department of Neurology, The First Bethune Hospital of Jilin University, Jilin University, Xinmin Street 71, Changchun 130021, China.
authors: Hongliang
authors: H
authors: Zhang
authors: Chunkui
authors: C
authors: Zhou
authors: Limin
authors: L
authors: Wu
authors: Fengming
authors: F
authors: Ni
authors: Jie
authors: J
authors: Zhu
authors: Tao
authors: T
authors: Jin
mesh_terms: D000818:Animals; D000912:Antibodies, Neoplasm / Q000276:immunology*; D000951:Antigens, Neoplasm / Q000276:immunology; D001921:Brain / Q000473:pathology; D002462:Cell Membrane / Q000276:immunology; D003937:Diagnosis, Differential; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007167:Immunotherapy / Q000379:methods; D020363:Limbic Encephalitis / Q000276:immunology* / Q000473:pathology* / Q000628:therapy; D008279:Magnetic Resonance Imaging
chemical_list: D000912:Antibodies, Neoplasm; D000951:Antigens, Neoplasm; D007074:Immunoglobulin G
references: Lancet. 1993 Jan 2;341(8836):21-2
references: 8093269
references: J Neuroimmunol. 2011 Mar;232(1-2):166-70
references: 21093932
references: Acta Neurol Scand Suppl. 2009;(189):63-7
references: 19566502
references: Brain Pathol. 1999 Apr;9(2):275-84
references: 10219745
references: Neurology. 2011 Nov 1;77(18):1698-701
references: 22013185
references: Curr Opin Neurol. 2007 Dec;20(6):732-7
references: 17992098
references: Lancet Neurol. 2010 Aug;9(8):776-85
references: 20580615
references: J Neurol Neurosurg Psychiatry. 2004 Aug;75(8):1135-40
references: 15258215
references: J Neurooncol. 2007 Jan;81(1):71-4
references: 16807779
references: J Neurol Neurosurg Psychiatry. 1948 May;11(2):73-87
references: 18861108
references: Acta Neuropathol. 2002 May;103(5):509-15
references: 11935268
references: Brain. 2000 Jul;123 ( Pt 7):1481-94
references: 10869059
references: J Clin Oncol. 1997 Aug;15(8):2866-72
references: 9256130
references: J Immunol. 1979 Apr;122(4):1548-55
references: 221577
references: N Engl J Med. 2000 Jan 6;342(1):21-7
references: 10620645
references: Lancet Neurol. 2008 Apr;7(4):327-40
references: 18339348
references: Mayo Clin Proc. 2003 Nov;78(11):1363-8
references: 14601695
references: Ann Neurol. 2007 Jun;61(6):544-51
references: 17600364
references: Epilepsy Behav. 2007 Jun;10(4):529-38
references: 17493878
references: J Neurol Neurosurg Psychiatry. 2007 Apr;78(4):381-5
references: 16980333
references: Ann Neurol. 1999 Feb;45(2):162-7
references: 9989617
references: Ann Neurol. 2000 Jan;47(1):9-17
references: 10632096
references: Neurology. 1994 Jan;44(1):140-7
references: 8290049
references: J Neurol Sci. 2004 May 15;220(1-2):85-8
references: 15140611
references: Brain. 2012 May;135(Pt 5):1622-38
references: 22539258
references: Gastroenterology. 2003 Jul;125(1):70-9
references: 12851872
references: Lancet Neurol. 2011 Jan;10(1):63-74
references: 21163445
references: Lancet Neurol. 2010 Jan;9(1):67-76
references: 19962348
references: Brain. 2001 Jun;124(Pt 6):1138-48
references: 11353730
references: Neurology. 2003 Jan 28;60(2):230-4
references: 12552036
references: Neurol Clin. 2003 Feb;21(1):221-47, ix
references: 12690651
references: Neurology. 2011 Mar 1;76(9):795-800
references: 21357831
references: Ann Neurol. 1984 Aug;16(2):193-208
references: 6383191
references: Neurology. 2008 Dec 2;71(23):1921-3
references: 19047564
references: Am J Med Sci. 2010 Oct;340(4):301-8
references: 20460982
references: Neurology. 1992 Mar;42(3 Pt 1):536-40
references: 1312683
references: Brain. 2004 Mar;127(Pt 3):701-12
references: 14960497
references: J Neurosci. 2010 Apr 28;30(17):5866-75
references: 20427647
references: J Neurol Neurosurg Psychiatry. 1997 Mar;62(3):276-8
references: 9069485
references: Brain. 2010 Sep;133(9):2734-48
references: 20663977
references: J Neurol. 2010 Oct;257(10):1686-91
references: 20517615
references: J Neurol Sci. 1999 Sep 15;168(1):28-31
references: 10500270
references: J Clin Pathol. 1997 Dec;50(12):975-80
references: 9516876
references: Brain. 1997 Jun;120 ( Pt 6):923-8
references: 9217677
references: Ann Neurol. 2010 Apr;67(4):470-8
references: 20437582
references: Cancer Immunol Immunother. 2009 Nov;58(11):1795-800
references: 19294382
references: Brain. 1968 Sep;91(3):481-96
references: 5723018
references: J Neurol Neurosurg Psychiatry. 2007 Apr;78(4):391-4
references: 17098840
references: Oncologist. 2006 Mar;11(3):292-305
references: 16549814
references: Brain. 2004 Aug;127(Pt 8):1831-44
references: 15215214
references: J Neurol. 1999 Apr;246(4):299-303
references: 10367699
references: Neuroradiology. 2011 Nov;53(11):837-51
references: 21271243
references: Curr Opin Neurol. 2010 Apr;23(2):144-50
references: 20164770
references: Ann Neurol. 2001 Feb;49(2):146-54
references: 11220734
references: Neurology. 1998 Oct;51(4):1146-50
references: 9781545
references: Lancet Neurol. 2008 Dec;7(12):1091-8
references: 18851928
references: Brain. 2010 Nov;133(11):3166-80
references: 20884644
references: Brain. 2005 Aug;128(Pt 8):1764-77
references: 15888538
references: Neurology. 2008 Sep 16;71(12):930-6
references: 18794496
references: Ann Neurol. 2009 Apr;65(4):424-34
references: 19338055
references: J Neurol. 2010 Apr;257(4):509-17
references: 20035430