文献詳細
増大特集 Antibody Update 2018
文献概要
電位依存性カリウムチャネル(VGKC)複合体抗体は,アイザックス症候群,モルヴァン症候群,さらに近時記憶障害とてんかんを主張とする辺縁系脳炎の病態関連抗体である。VGKC複合体の主要抗原はLGI1(leucine-rich glioma inactivated protein 1)とCaspr2(contactin-associated protein 2)である。これらに対する抗体が陰性の場合,VGKC複合体抗体が細胞内ドメインに反応しており,病態には直接関連はない。
参考文献
1)Hart IK, Waters C, Vincent A, Newland C, Beeson D, et al: Autoantibodies detected to expressed K+ channels are implicated in neuromyotonia. Ann Neurol 41: 238-246, 1997
2)Hart IK, Maddison P, Newsom-Davis J, Vincent A, Mills KR: Phenotypic variants of autoimmune peripheral nerve hyperexcitability. Brain 125: 1887-1895, 2002
3)Vincent A, Jacobson L, Plested P, Polizzi A, Tang T, et al: Antibodies affecting ion channel function in acquired neuromyotonia, in seropositive and seronegative myasthenia gravis, and in antibody-mediated arthrogryposis multiplex congenita. Ann N Y Acad Sci 841: 482-496, 1998
4)Maddison P, Mills KR, Newsom-Davis J: Clinical electrophysiological characterization of the acquired neuromyotonia phenotype of autoimmune peripheral nerve hyperexcitability. Muscle Nerve 33: 801-808, 2006
5)Gantenbein AR, Wiederkehr M, Meuli-Simmen C, Schwegler G: Focal neuromyotonia: do I love you? J Neurol 257: 1727-1729, 2010
channels of peripheral nerves. Ann Neurol 38: 714-722, 1995
7)渡邊 修, 中村友紀, 道園久美子, 松浦英治, 髙嶋 博: Isaacs症候群における早期診断基準の妥当性に関する検証. 厚生労働省難治性疾患等克服研究事業「神経免疫疾患のエビデンスによる診断基準・重症度分類・ガイドラインの妥当性と患者QOLの検証研究」班 平成29年度報告書.[印刷中]
8)Arimura K, Ng A, Watanabe O: Immune-mediated potassium channelopathies. Barber C, Tsuji S, Tobimatsu S, Uozumi T, Akamatsu N, et al (eds): Functional Neuroscience: Evoked Potentials and Related Techniques. Elsevier, Tokyo, 2006, pp269-275
9)Ahmed A, Simons Z: Isaacs syndrome: a review. Muscle Nerve 52: 5-12, 2015
10)Isaacs H: A syndrome of continuous muscle-fibre activity. J Neurol Neurosurg Psychiatry 24: 319-325, 1961
11)Sonoda Y, Arimura K, Kurono A, Suehara M, Kameyama M, et al: Serum of Isaacs' syndrome suppresses potassium channels in PC-12 cell lines. Muscle Nerve 19: 1439-1446, 1996
12)Nagado T, Arimura K, Sonoda Y, Kurono A, Horikiri Y, et al: Potassium current suppression in patients with peripheral nerve hyperexcitability. Brain 122: 2057-2066, 1999
channel antibodies in acquired neuromyotonia. Ann Neurol 56: 440-444, 2004
14)Walusinski O, Honnorat J: Augustin Morvan (1819-1897), a little-known rural physician and neurologist. Rev Neurol (Paris) 169: 2-8, 2013
15)Liguori R, Vincent A, Clover L, Avoni P, Plazzi G, et al: Morvan's syndrome: peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels. Brain 124: 2417-2426, 2001
16)Irani SR, Pettingill P, Kleopa K, Schiza N, Waters P, et al: Morvan syndrome: clinical and serological observations in 29 cases. Ann Neurol 72: 241-255, 2012
17)Klein CJ, Lennon VA, Aston PA, McKeon A, Pittock SJ: Chronic pain as a manifestation of potassium channel-complex autoimmunity. Neurology 79: 1136-1144, 2012
18)Sharma S, Sharma P: Morvan syndrome: after scrotal sac drainage and chemical instillation in hydrocele. Neurol India 61: 300-302, 2013
channels. Neuron 24: 1037-1047, 1999
20)Buckley C, Oger J, Clover L, Tüzün E, Carpenter K, et al: Potassium channel antibodies in two patients with reversible limbic encephalitis. Ann Neurol 50: 73-78, 2001
21)Vincent A, Buckley C, Schott JM, Baker I, Dewar BK, et al: Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 127: 701-712, 2004
22)Thieben MJ, Lennon VA, Boeve BF, Aksamit AJ, Keegan M, et al: Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. Neurology 62: 1177-1182, 2004
23)Huda S, Wong SH, Pettingill P, O'Connell D, Vincent A, et al: An 11-year retrospective experience of antibodies against the voltage-gated potassium channel (VGKC) complex from a tertiary neurological centre. J Neurol 262: 418-424, 2015
24)Lancaster E, Hernandez EM, Dalmau J: Encephalitis and antibodies to synaptic and neuronal cell surface protein. Neurology 77: 179-189, 2011
25)Quek AM, Britton JW, McKeon A, So E, Lennon VA, et al: Autoimmune epilepsy: clinical characteristics and response to immunotherapy. Arch Neurol 69: 582-593, 2012
26)Malter MP, Helmstaedter C, Urbach H, Vincent A, Bien CG: Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis. Ann Neurol 67: 470-478, 2010
27)Irani SR, Michell AW, Lang B, Pettingill P, Waters P, et al: Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol 69: 892-900, 2011
28)Naasan G, Irani SR, Bettcher BM, Geschwind MD, Gelfand JM: Episodic bradycardia as neurocardiac prodrome to voltage-gated potassium channel complex/leucine-rich, glioma inactivated 1 antibody encephalitis. JAMA Neurol 71: 1300-1304, 2014
29)Willis MD, Jones L, Vincent A, Wheeler R, O'Callaghan P, et al: VGKC-complex antibody encephalitis. QJM 107: 657-659, 2014
30)Wieser S, Kelemen A, Barsi P, Vincent A, Borbely C, et al: Pilomotor seizures and status in non-paraneoplastic limbic encephalitis. Epileptic Disord 7: 205-211, 2005
31)Rocamora R, Becerra JL, Fossas P, Gomez M, Vivanco-Hidalgo RM, et al: Pilomotor seizures: an autonomic semiology of limbic encephalitis? Seizure 23: 670-673, 2014
32)Boesebeck F, Schwarz O, Dohmen B, Graef U, Vestring T, et al: Faciobrachial dystonic seizures arise from cortico-subcortical abnormal brain areas. J Neurol 260: 1684-1686, 2013
33)Bien CG, Vincent A, Barnett MH, Becker AJ, Blümcke I, et al: Immunopathology of autoantibody-associated encephalitides: clues for pathogenesis. Brain 135: 1622-1638, 2012
34)Butler CR, Miller TD, Kaur MS, Baker IW, Boothroyd GD, et al: Persistent anterograde amnesia following limbic encephalitis associated with antibodies to the voltage-gated potassium channel complex. J Neurol Neurosurg Psychiatry 85: 387-391, 2014
35)Ohkawa T, Fukata Y, Yamasaki M, Miyazaki T, Yokoi N, et al: Autoantibodies to epilepsy-related LGI1 in limbic encephalitis neutralize LGI1-ADAM22 interaction and reduce synaptic AMPA receptors. J Nuerosci 33: 18161-18174, 2013
receptor autoantibodies in autoimmune encephalitis. J Neurosci 34: 8151-8163, 2014
37)Irani SR, Stagg CJ, Schott JM, Rosenthal CR, Schneider SA, et al: Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype. Brain 136: 3151-3162, 2013
38)Irani SR, Gelfand JM, Al-DiwaniA Vincent A: Cell-surface central nervous system autoantibodies: clinical relevance and emerging paradigms. Ann Neurol 76: 168-184, 2014
39)Szots M, Marton A, Kover F, Kiss T, Berki T, et al: Natural course of LGI1 encephalitis: 3-5 years of follow-up without immunotherapy. J Neurol Sci 343: 198-202, 2014
40)Tan KM, Lennon VA, Klein CJ, Boeve BF, Pittock SJ: Clinical spectrum of voltage-gated potassium channel autoimmunity. Neurology 70: 1883-1890, 2008
41)Geschwind MD, Tan KM, Lennon VA, Barajas RF Jr, Haman A, et al: Voltage-gated potassium channel autoimmunity mimicking Creutzfeldt-Jakob Disease. Arch Neurol 65: 1341-1346, 2008
42)Rossi M, Mead S, Collinge J, Rudge P, Vincent A: Neuronal antibodies in patients with suspected or confirmed sporadic Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 86: 692-694, 2015
43)Nwosu VK, Royer JA, Stickler DE: Voltage gated potassium channel antibodies in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 11: 392-394, 2010
44)佐藤 晶, 酒井直子, 新保淳輔, 橋立英樹, 五十嵐修一, 他: 著明な筋痙攣・線維束性収縮をともない抗voltage gated potassium channel(VGKC)複合体抗体が高値であった筋萎縮性側索硬化症の1剖検例. 臨床神経54: 32-37, 2014
45)Donaldson R, Li J, Li Y: Clinical significance of cation channel antibodies in motor neuron disease. Muscle Nerve 54: 228-231, 2016
46)Lang B, Makuch M, Moloney T, Dettmann I, Mindorf S, et al: Intracellular and non-neuronal targets of voltage-gated potassium channel complex antibodies. J Neurol Neurosurg Psychiatry 88: 353-361, 2017
47)Binks SNM, Klein CJ, Waters P, Pittock SJ, Irani SR: LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes. J Neurol Neurosurg Psychiatry. 2017 Oct 21.[Epub ahead of print][doi: 10.1136/jnnp-2017-315720]
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