Review Article Nodopathies: A distinct pathophysiological and clinical entity
Nodopathies: A distinct pathophysiological and clinical entity
DOI:
https://doi.org/10.15713/ins.bhj.74Abstract
Abstract
In recent years, antibodies directed against the proteins located at the node of Ranvier have been identified in patients with immune mediated neuropathies. In this article, we review the structure of node of Ranvier and pathology associated with these antibody mediated neuropathies. We summarize the emerging concept of nodopathy/ paranodopathy, its clinical relevance and treatment implications.
References
Ranvier L Contributions à l'histologie et à la physiologie des nerfs périphériques. C R Acad Sci. 1871; 73: 1168-1171.
A. F. Huxley and R. Stämpfli. Evidence for saltatory conduction in peripheral myelinated nerve fibres. J Physiol. 1949 May 15; 108(3): 315–339.
Antonino Uncini, Satoshi Kuwabara. Nodopathies of the peripheral nerve: an emerging concept. J Neurol Neurosurg Psychiatry 2015 Nov;86(11):1186-95.
Antonino Uncini, Keiichira Susuki, Nobuhira Yuki. Nodo-paranodopathy: Beyond the demyelinating and axonal classification in anti-ganglioside antibody-mediated neuropathies. Clinical Neurophysiology 2013 Oct;124(10):1928-34.
Isabel Illa. ARTHUR ASBURY LECTURE: Chronic inflammatory demyelinating polyradiculoneuropathy: clinical aspects and new animal models of auto-immunity to nodal components. J Peripher Nerv Syst 2017 Dec;22(4):418-424.
Atay Vural, Kathrin Doppler and Edgar Meinl. Autoantibodies against the node of Ranvier in seropositive chronic inflammmatory demyelinating polyneuropathy: Diagnostic, pathogenic, and therapeutic relevance. Front Immunol 2018 May 14;9:1029.
Kuwabara S, Yuki N, Koga M, Hattori T, Matsuura D, Miyake M, Noda M. IgG anti-GM1 antibody is associated with reversible conduction failure and axonal degeneration in Guillain-Barré syndrome. Ann Neurol 1998: 44:202-208.
Shin J Oh. Nodal conduction block: A unifying concept. Muscle Nerve 2021 Feb;63(2):178-180.
Hughes RAC, Comblath DR. Gullain-Barre syndrome. Lancet 2005;366:1653-66.
Koike H and Katsuno M. Macrophages and autoantibodies in demyelinating diseases. Cells 2021Apr 8;10(4):844.
Vlam L, van der Pol WL, Cats EA, et al. Multifocal motor neuropathy: diagnosis, pathogenesis and treatment strategies. Nat Rev Neurol 2011;8:48–58.
Uncini A, Santoro M, Corbo M, Lugaresi A, Latov N. Conduction abnormalities induced by sera of patients with multifocal motor neuropathy and anti-GM1 antibodies. Muscle Nerve 1993;16:610–15.
Kiernan MC, Guglielmi JM, Kaji R, Murray NMF, Bostock H. Evidence for axonal membrane hyperpolarization in multifocal motor neuropathy with conduction block. Brain 2002;125:664–75.
Anna Hiu, Yi Wong and Nobuhiro Yuki. Autoimmune inflammatory neuropathies: updates in pathogenesis, diagnosis and treatment. Curr Opin Neurol 2015; 28:468-473.
Bunschoten C, Jacobs BC, Van den Bergh PYK, Cornblath DR, van Doorn PA. Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Lancet Neurol 2019; 18(8):784-794.
Querol L, Nogales-Gadea G, Rojas-Garcia R, Martinez-Hernandez E, Diaz-Manera J, Suarez-Calvet X, et al. Antibodies to contactin-1 in chronic inflammatory demyelinating polyneuropathy. Ann Neurol 2013 Mar;73(3):370-80.
Querol L, Devaux J, Rojas-Garcia R, Illa I. Autoantibodies in chronic inflammatory neuropathies: diagnostic and therapeutic implications. Nat Rev Neurol 2017;13(9):533-547.
Miura Y, Devaux JJ, Fukami Y, Manso C, Belghazi M, Wong AH, Yuki N. Contactin1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia. Brain 2015 Jun;138(Pt 6):1484-91.
Koike H, Kadoya M, Kaida K, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima et al. Paranodal dissection in chronic inflammatory demyelinating neuropathy with anti-neurofascin 155 and anticontactin 1 antibodies. J Neurol Neurosurg Psychiatry. 2017 Jun;88(6):465-473.
Manso C, Querol L, Mekaouche M, Illa I, Devaux JJ. Contactin-1 IgG4 antibodies cause paranode dismantling and conduction defects. Brain 2016 Jun;139(Pt 6):1700-12.
Ng JK, Malotka J, Kawakami N, Derfuss T, Khademi M, Olsson T, et al. Neurofascin as a target for autoantibodies in peripheral neuropathies. Neurology 2012 Dec 4;79(23):2241-8.
Querol L, Nogales-Gadea G, Rojas-Garcia R, Diaz-Manera J, Pardo J, Ortega-Moreno A, et al. Neurofascin IgG4 antibodies in CIDP associate with disabling tremor and poor response to IVIg. Neurology 2014 Mar 11;82(10):879-86.
Ogata H, Yamasaki R, Hiwatashi A, Oka N, Kawamura N, Matsuse D, et al. Characterization of IgG4 anti-neurofascin 155 antibody-positive polyneuropathy. Ann Clin Transl Neurol 2015 Oct;2(10):960-71.
Jun-ichi Kira, Ryo Yamasaki, Hidenori Ogata. Anti-neurofascin autoantibody and demyelination. Neurochem Int. 2019 Nov;130:104360.
Manso C, Querol L, Mekaouche M, Illa I, Devaux J (2017). Anti- NFASC155 IGG4 affects paranode structure in animal models In: PNS Annual Meeting. Peripheral Nerve Society, Sitges, Spain, p 221.
Doppler K, Appeltshauser L, Villmann C, Martin C, Peles E, Kramer HH, et al. Auto-antibodies to contactin-associated protein 1 (Caspr) in two patients with painful inflammatory neuropathy. Brain 139:2617-2630.
Delmont E, Manso C, Querol L, Berardinelli A, Lozza A, Belghazi M, et al. Autoantibodies to nodal isoforms of neurofascin in chronic inflammatory demyelinating polyneuropathy. Brain 2017; 140; 1851-8.
Kazim A. Sheikh. Guillain-Barre Syndrome. Continuum October 2020; Vol 26 No.5: 1184-1204.
Misawa S, Kuwabara S, Sato Y, Yamaguchi N, Nagashima K, Katayama K, et al. Safety and efficacy of eculizumab in Guillain-Barre syndrome: a multicentre, double-blind, randomised phase 2 trial. Lancet Neurol. 2018 Jun;17(6):519-529.
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