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Chapter 1 - Importance, Definitions, History, Classification, and Frequency of the Autoimmune Encephalitides

from Section 1 - Overview

Published online by Cambridge University Press:  27 January 2022

Josep Dalmau  and
Francesc Graus
Josep Dalmau
Affiliation:
Universitat de Barcelona
Francesc Graus
Affiliation:
Universitat de Barcelona
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Summary

About 20 years ago the group of diseases currently known as ‘autoimmune encephalitis’ or ‘antibody-mediated encephalitis’ was unknown and the entire field of ‘autoimmune neurology’ non-existent. Since then, 18 autoimmune encephalitis and the corresponding syndromes have been described, including 16 in which the antigens are expressed on the cell surface of neurons and two on the surface of glial cells. The characterization of these autoimmune encephalitis was facilitated by the cumulative knowledge provided by research on autoimmune disorders of the neuromuscular junction (myasthenia gravis and Lambert–Eaton myasthenic syndrome) and the paraneoplastic neurological syndromes. Up to 12.6 per 100,000 persons are affected by encephalitis annually. Of these, it has been estimated that 20–30% are caused by autoimmune mechanisms. In children the most frequent types of autoimmune encephalitis are acute disseminated encephalomyelitis (ADEM), anti-MOG, and anti-NMDAR encephalitis. In young adults, particularly women, anti-NMDAR encephalitis, and in late adulthood, anti-LGI1 encephalitis, are the most prevalent autoimmune encephalitis. The most frequently used classifications combine information related to three features: mechanisms of disease (cytotoxic T cell or antibody-mediated mechanisms), type of antigen (intracellular vs cell surface), and presence or absence of a tumour. The detection of a neoplasm frequently serves to categorize the autoimmune encephalitis as paraneoplastic.

Keywords

Autoimmune encephalitisNMDAR antibodiesantibodies against synaptic receptorsonconeural antibodieslimbic encephalitisparaneoplastic neurological syndromes
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Autoimmune Encephalitis and Related Disorders of the Nervous System , pp. 1 - 18
Publisher: Cambridge University Press
Print publication year: 2022

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References

Dalmau, J, Geis, C, Graus, F. Autoantibodies to synaptic receptors and neuronal cell surface proteins in autoimmune diseases of the central nervous system. Physiol Rev 2017;97:839–887.CrossRefGoogle ScholarPubMed
Jarius, S, Paul, F, Aktas, O, et al. MOG encephalomyelitis: international recommendations on diagnosis and antibody testing. J Neuroinflammation 2018;15:134.Google Scholar
Zekeridou, A, Lennon, VA. Aquaporin-4 autoimmunity. Neurol Neuroimmunol Neuroinflamm 2015;2:e110.Google Scholar
Sabater, L, Gaig, C, Gelpi, E, et al. A novel non-rapid-eye movement and rapid-eye-movement parasomnia with sleep breathing disorder associated with antibodies to IgLON5: a case series, characterisation of the antigen, and post-mortem study. Lancet Neurol 2014;13:575–586.CrossRefGoogle ScholarPubMed
Graus, F, Titulaer, MJ, Balu, R, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016;15:391–404.CrossRefGoogle ScholarPubMed
Venkatesan, A, Michael, BD, Probasco, JC, Geocadin, RG, Solomon, T. Acute encephalitis in immunocompetent adults. Lancet 2019;393:702–716.Google Scholar
Iizuka, T, Sakai, F, Ide, T, et al. Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal. Neurology 2008;70:504–511.Google Scholar
Parratt, KL, Allan, M, Lewis, SJ, et al. Acute psychiatric illness in a young woman: an unusual form of encephalitis. Med J Aust 2009;191:284–286.CrossRefGoogle Scholar
Darnell, RB, Posner, JB. Paraneoplastic syndromes. New York: Oxford University Press, 2011.Google Scholar
Brierley, JB, Corsellis, JAN, Hierons, R, et al. Subacute encephalitis of later adult life: mainly affecting the limbic areas. Brain 1960;83:357–368.Google Scholar
Corsellis, JA, Goldberg, GJ, Norton, AR. ‘Limbic encephalitis’ and its association with carcinoma. Brain 1968;91:481–496.Google Scholar
Dalmau, J, Graus, F. Antibody-mediated encephalitis. N Engl J Med 2018;378:840–851.Google Scholar
Irani, SR, Alexander, S, Waters, P, et al. Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan’s syndrome and acquired neuromyotonia. Brain 2010;133:2734–2748.CrossRefGoogle ScholarPubMed
Lai, M, Huijbers, MG, Lancaster, E, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol 2010;9:776–785.Google Scholar
Lai, M, Hughes, EG, Peng, X, et al. AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol 2009;65:424–434.Google Scholar
Lancaster, E, Lai, M, Peng, X, et al. Antibodies to the GABA(B) receptor in limbic encephalitis with seizures: case series and characterisation of the antigen. Lancet Neurol 2010;9:67–76.CrossRefGoogle Scholar
Hoftberger, R, von Sonderen, A, Leypoldt, F, et al. Encephalitis and AMPA receptor antibodies: novel findings in a case series of 22 patients. Neurology 2015;84:2403–2412.Google Scholar
Hoftberger, R, Titulaer, MJ, Sabater, L, et al. Encephalitis and GABAB receptor antibodies: novel findings in a new case series of 20 patients. Neurology 2013;81:1500–1506.CrossRefGoogle Scholar
Dalmau, J, Gleichman, AJ, Hughes, EG, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008;7:1091–1098.Google Scholar
Spatola, M, Sabater, L, Planaguma, J, et al. Encephalitis with mGluR5 antibodies: symptoms and antibody effects. Neurology 2018;90:e1964–e1972.Google Scholar
Dalmau, J, Furneaux, HM, Cordon-Cardo, C, Posner, JB. The expression of the Hu (paraneoplastic encephalomyelitis/sensory neuronopathy) antigen in human normal and tumor tissues. Am J Pathol 1992;141:881–886.Google ScholarPubMed
Small, M, Treilleux, I, Couillault, C, et al. Genetic alterations and tumor immune attack in Yo paraneoplastic cerebellar degeneration. Acta Neuropathol 2018;135:569–579.Google Scholar
Ariño, H, Hoftberger, R, Gresa-Arribas, N, et al. Paraneoplastic neurological syndromes and glutamic acid decarboxylase antibodies. JAMA Neurol 2015;72:874–881.Google Scholar
Do, LD, Chanson, E, Desestret, V, et al. Characteristics in limbic encephalitis with anti-adenylate kinase 5 autoantibodies. Neurology 2017;88:514–524.CrossRefGoogle ScholarPubMed
Russell, DS. Encephalomyelitis and carcinomatous neuropathy. In: van Bogaert, L, Radermecker, J, Hozay, J, Lowenthal, A, eds. The encephalitides. Amsterdam: Elsevier, 1961: 131–135.Google Scholar
Wilkinson, PC, Zeromski, J. Immunofluorescent detection of antibodies against neurons in sensory carcinomatous neuropathy. Brain 1965;88:529–538.CrossRefGoogle ScholarPubMed
Horwich, MS, Cho, L, Porro, RS, Posner, JB. Subacute sensory neuropathy: a remote effect of carcinoma. Ann Neurol 1977;2:7–19.CrossRefGoogle ScholarPubMed
Graus, F, Cordon-Cardo, C, Posner, JB. Neuronal antinuclear antibody in sensory neuronopathy from lung cancer. Neurology 1985;35:538–543.Google Scholar
Graus, F, Elkon, KB, Cordon-Cardo, C, Posner, JB. Sensory neuronopathy and small cell lung cancer: antineuronal antibody that also reacts with the tumor. Am J Med 1986;80:45–52.Google Scholar
Dalmau, J, Graus, F, Rosenblum, MK, Posner, JB. Anti-Hu–associated paraneoplastic encephalomyelitis/sensory neuronopathy: a clinical study of 71 patients. Medicine (Baltimore) 1992;71:59–72.Google Scholar
Trotter, JL, Hendin, BA, Osterland, CK. Cerebellar degeneration with Hodgkin disease. An immunological study. Arch Neurol 1976;33:660–661.Google Scholar
Graus, F, Dalmau, J, Valldeoriola, F, et al. Immunological characterization of a neuronal antibody (anti-Tr) associated with paraneoplastic cerebellar degeneration and Hodgkin’s disease. J Neuroimmunol 1997;74:55–61.CrossRefGoogle ScholarPubMed
de Graaff, E, Maat, P, Hulsenboom, E, et al. Identification of delta/notch-like epidermal growth factor-related receptor as the Tr antigen in paraneoplastic cerebellar degeneration. Ann Neurol 2012;71:815–824.CrossRefGoogle ScholarPubMed
Greenlee, JE, Brashear, HR. Antibodies to cerebellar Purkinje cells in patients with paraneoplastic cerebellar degeneration and ovarian carcinoma. Ann Neurol 1983;14:609–613.Google Scholar
Jaeckle, KA, Graus, F, Houghton, A, et al. Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen. Ann Neurol 1985;18:592–600.Google Scholar
Graus, F, Delattre, JY, Antoine, JC, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 2004;75:1135–1140.Google Scholar
Graus, F, Vega, F, Delattre, JY, et al. Plasmapheresis and antineoplastic treatment in CNS paraneoplastic syndromes with antineuronal autoantibodies. Neurology 1992;42:536–540.CrossRefGoogle ScholarPubMed
Uchuya, M, Graus, F, Vega, F, Reñé, R, Delattre, JY. Intravenous immunoglobulin treatment in paraneoplastic neurological syndromes with antineuronal autoantibodies. J Neurol Neurosurg Psychiatry 1996;60:388–392.Google Scholar
Graus, F, Illa, I, Agusti, M, Ribalta, T, Cruz-Sanchez, F. Effect of intraventricular injection of an anti-Purkinje cell antibody (anti-Yo) in a guinea pig model. J Neurol Sci 1991;106:82–87.Google Scholar
Tanaka, M, Tanaka, K, Onodera, O, Tsuji, S. Trial to establish an animal model of paraneoplastic cerebellar degeneration with anti-Yo antibody. 1. Mouse strains bearing different MHC molecules produce antibodies on immunization with recombinant Yo protein, but do not cause Purkinje cell loss. Clin Neurol Neurosurg 1995;97:95–100.CrossRefGoogle Scholar
Simpson, JA. Myasthenia gravis, a new hypothesis. Scott Med J 1960;5:419–436.Google Scholar
Patrick, J, Lindstrom, J. Autoimmune response to acetylcholine receptor. Science 1973;180:871–872.Google Scholar
Toyka, KV, Brachman, DB, Pestronk, A, Kao, I. Myasthenia gravis: passive transfer from man to mouse. Science 1975;190:397–399.Google Scholar
Lindstrom, JM, Engel, AG, Seybold, ME, Lennon, VA, Lambert, EH. Pathological mechanisms in experimental autoimmune myasthenia gravis. II. Passive transfer of experimental autoimmune myasthenia gravis in rats with anti-acetylcholine receptor antibodies. J Exp Med 1976;144:739–753.Google Scholar
Drachman, DB, Angus, CW, Adams, RN, Michelson, JD, Hoffman, GJ. Myasthenic antibodies cross-link acetylcholine receptors to accelerate degradation. N Engl J Med 1978;298:1116–1122.Google Scholar
Hughes, EG, Peng, X, Gleichman, AJ, et al. Cellular and synaptic mechanisms of anti-NMDA receptor encephalitis. J Neurosci 2010;30:5866–5875.Google Scholar
Engel, AG, Lindstrom, JM, Lambert, EH, Lennon, VA. Ultrastructural localization of the acetylcholine receptor in myasthenia gravis and in its experimental autoimmune model. Neurology 1977;27:307–315.Google Scholar
Hoch, W, McConville, J, Helms, S, et al. Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med 2001;7:365–368.Google Scholar
Klooster, R, Plomp, JJ, Huijbers, MG, et al. Muscle-specific kinase myasthenia gravis IgG4 autoantibodies cause severe neuromuscular junction dysfunction in mice. Brain 2012;135:1081–1101.Google Scholar
Diaz-Manera, J, Martinez-Hernandez, E, Querol, L, et al. Long-lasting treatment effect of rituximab in MuSK myasthenia. Neurology 2012;78:189–193.Google Scholar
Huijbers, MG, Zhang, W, Klooster, R, et al. MuSK IgG4 autoantibodies cause myasthenia gravis by inhibiting binding between MuSK and Lrp4. Proc Natl Acad Sci U S A 2013;110:20783–20788.Google Scholar
Lennon, VA, Lambert, EH, Whittingham, S, Fairbanks, V. Autoimmunity in the Lambert–Eaton myasthenic syndrome. Muscle Nerve 1982;5:S21–25.Google Scholar
Lang, B, Newsom-Davis, J, Wray, D, Vincent, A, Murray, N. Autoimmune aetiology for myasthenic (Eaton–Lambert) syndrome. Lancet 1981;2:224–226.CrossRefGoogle ScholarPubMed
Lang, B, Newsom-Davis, J, Prior, C, Wray, D. Antibodies to motor nerve terminals: an electrophysiological study of a human myasthenic syndrome transferred to mouse. J Physiol 1983;344:335–345.Google Scholar
Kim, YI. Passive transfer of the Lambert–Eaton myasthenic syndrome: neuromuscular transmission in mice injected with plasma. Muscle Nerve 1985;8:162–172.CrossRefGoogle ScholarPubMed
Fukunaga, H, Engel, AG, Lang, B, Newsom-Davis, J, Vincent, A. Passive transfer of Lambert–Eaton myasthenic syndrome with IgG from man to mouse depletes the presynaptic membrane active zones. Proc Natl Acad Sci U S A 1983;80:7636–7640.Google Scholar
Fukuoka, T, Engel, AG, Lang, B, et al. Lambert–Eaton myasthenic syndrome: I. Early morphological effects of IgG on the presynaptic membrane active zones. Ann Neurol 1987;22:193–199.Google Scholar
Fukuoka, T, Engel, AG, Lang, B, Newsom-Davis, J, Vincent, A. Lambert–Eaton myasthenic syndrome: II. Immunoelectron microscopy localization of IgG at the mouse motor end-plate. Ann Neurol 1987;22:200–211.Google Scholar
Roberts, A, Perera, S, Lang, B, Vincent, A, Newsom-Davis, J. Paraneoplastic myasthenic syndrome IgG inhibits 45Ca2+ flux in a human small cell carcinoma line. Nature 1985;317:737–739.Google Scholar
Sher, E, Gotti, C, Canal, N, et al. Specificity of calcium channel autoantibodies in Lambert–Eaton myasthenic syndrome. Lancet 1989;2:640–643.Google Scholar
Motomura, M, Johnston, I, Lang, B, Vincent, A, Newsom-Davis, J. An improved diagnostic assay for Lambert–Eaton myasthenic syndrome. J Neurol Neurosurg Psychiatry 1995;58:85–87.Google Scholar
Lennon, VA, Kryzer, TJ, Griesmann, GE, et al. Calcium-channel antibodies in the Lambert–Eaton syndrome and other paraneoplastic syndromes. N Engl J Med 1995;332:1467–1474.Google Scholar
Halbach, M, Homberg, V, Freund, HJ. Neuromuscular, autonomic and central cholinergic hyperactivity associated with thymoma and acetylcholine receptor-binding antibody. J Neurol 1987;234:433–436.CrossRefGoogle ScholarPubMed
Waerness, E. Neuromyotonia and bronchial carcinoma. Electromyogr Clin Neurophysiol 1974;14:527–535.Google Scholar
Partanen, VS, Soininen, H, Saksa, M, Riekkinen, P. Electromyographic and nerve conduction findings in a patient with neuromyotonia, normocalcemic tetany and small-cell lung cancer. Acta Neurol Scand 1980;61:216–226.CrossRefGoogle Scholar
Reeback, J, Benton, S, Swash, M, Schwartz, MS. Penicillamine-induced neuromyotonia. Br Med J 1979;1:1464–1465.Google Scholar
Sinha, S, Newsom-Davis, J, Mills, K, et al. Autoimmune aetiology for acquired neuromyotonia (Isaacs’ syndrome). Lancet 1991;338:75–77.CrossRefGoogle ScholarPubMed
Shillito, P, Molenaar, PC, Vincent, A, et al. Acquired neuromyotonia: evidence for autoantibodies directed against K+ channels of peripheral nerves. Ann Neurol 1995;38:714–722.Google Scholar
Hart, IK, Maddison, P, Newsom-Davis, J, Vincent, A, Mills, KR. Phenotypic variants of autoimmune peripheral nerve hyperexcitability. Brain 2002;125:1887–1895.Google Scholar
Lancaster, E, Huijbers, MG, Bar, V, et al. Investigations of caspr2, an autoantigen of encephalitis and neuromyotonia. Ann Neurol 2011;69:303–311.Google Scholar
Rubio-Agusti, I, Perez-Miralles, F, Sevilla, T, et al. Peripheral nerve hyperexcitability: a clinical and immunologic study of 38 patients. Neurology 2011;76:172–178.CrossRefGoogle ScholarPubMed
Alamowitch, S, Graus, F, Uchuya, M, et al. Limbic encephalitis and small cell lung cancer: clinical and immunological features. Brain 1997;120:923–928.CrossRefGoogle ScholarPubMed
Castillo, P, Woodruff, B, Caselli, R, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol 2006;63:197–202.Google Scholar
Dale, RC, Church, AJ, Surtees, RA, et al. Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity. Brain 2004;127:21–33.Google Scholar
Madrid, A, Gil-Peralta, A, Gil-Neciga, E, Gonzalez, JR, Jarrin, S. Morvan’s fibrillary chorea: remission after plasmapheresis. J Neurol 1996;243:350–353.Google Scholar
Lee, EK, Maselli, RA, Ellis, WG, Agius, MA. Morvan’s fibrillary chorea: a paraneoplastic manifestation of thymoma. J Neurol Neurosurg Psychiatry 1998;65:857–862.Google Scholar
Antoine, JC, Honnorat, J, Anterion, CT, et al. Limbic encephalitis and immunological perturbations in two patients with thymoma. J Neurol Neurosurg Psychiatry 1995;58:706–710.Google Scholar
Liguori, R, Vincent, A, Clover, L, et al. Morvan’s syndrome: peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels. Brain 2001;124:2417–2426.Google Scholar
Buckley, C, Oger, J, Clover, L, et al. Potassium channel antibodies in two patients with reversible limbic encephalitis. Ann Neurol 2001;50:73–78.Google Scholar
Vincent, A, Buckley, C, Schott, JM, et al. Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 2004;127:701–712.Google Scholar
Kleopa, KA, Elman, LB, Lang, B, Vincent, A, Scherer, SS. Neuromyotonia and limbic encephalitis sera target mature Shaker-type K+ channels: subunit specificity correlates with clinical manifestations. Brain 2006;129:1570–1584.Google Scholar
Ances, BM, Vitaliani, R, Taylor, RA, et al. Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates. Brain 2005;128:1764–1777.Google Scholar
Vitaliani, R, Mason, W, Ances, B, et al. Paraneoplastic encephalitis, psychiatric symptoms, and hypoventilation in ovarian teratoma. Ann Neurol 2005;58:594–604.Google Scholar
Dalmau, J, Tuzun, E, Wu, HY, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 2007;61:25–36.Google Scholar
Szabo, A, Dalmau, J, Manley, G, et al. HuD, a paraneoplastic encephalomyelitis antigen, contains RNA-binding domains and is homologous to Elav and Sex-lethal. Cell 1991;67:325–333.Google Scholar
Sakai, K, Mitchell, DJ, Tsukamoto, T, Steinman, L. Isolation of a complementary DNA clone encoding an autoantigen recognized by an anti-neuronal cell antibody from a patient with paraneoplastic cerebellar degeneration [published erratum appears in Ann Neurol 1991 Nov;30(5):738]. Ann Neurol 1990;28:692–698.CrossRefGoogle ScholarPubMed
Buckanovich, RJ, Posner, JB, Darnell, RB. Nova, the paraneoplastic Ri antigen, is homologous to an RNA-binding protein and is specifically expressed in the developing motor system. Neuron 1993;11:657–672.Google Scholar
Voltz, R, Gultekin, SH, Rosenfeld, MR, et al. A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer [see comments]. N Engl J Med 1999;340:1788–1795.Google Scholar
Dropcho, EJ, Chen, YT, Posner, JB, Old, LJ. Cloning of a brain protein identified by autoantibodies from a patient with paraneoplastic cerebellar degeneration. Proc Natl Acad Sci U S A 1987;84:4552–4556.Google Scholar
Hutchinson, M, Waters, P, McHugh, J, et al. Progressive encephalomyelitis, rigidity, and myoclonus: a novel glycine receptor antibody. Neurology 2008;71:1291–1292.Google Scholar
Granerod, J, Ambrose, HE, Davies, NW, et al. Causes of encephalitis and differences in their clinical presentations in England: a multicentre, population-based prospective study. Lancet Infect Dis 2010;10:835–844.Google Scholar
Granerod, J, Cousens, S, Davies, NW, Crowcroft, NS, Thomas, SL. New estimates of incidence of encephalitis in England. Emerg Infect Dis 2013;19:1455–1462.Google Scholar
Kayser, MS, Titulaer, MJ, Gresa-Arribas, N, Dalmau, J. Frequency and characteristics of isolated psychiatric episodes in anti-N-methyl-d-aspartate receptor encephalitis. JAMA Neurol 2013;70:1133–1139.Google Scholar
Graus, F, Boronat, A, Xifro, X, et al. The expanding clinical profile of anti-AMPA receptor encephalitis. Neurology 2010;74:857–859.Google Scholar
Niehusmann, P, Dalmau, J, Rudlowski, C, et al. Diagnostic value of N-methyl-D-aspartate receptor antibodies in women with new-onset epilepsy. Arch Neurol 2009;66:458–464.Google Scholar
Thompson, J, Bi, M, Murchison, AG, et al. The importance of early immunotherapy in patients with faciobrachial dystonic seizures. Brain 2018;141:348–356.Google Scholar
Ariño, H, Armangue, T, Petit-Pedrol, M, et al. Anti-LGI1-associated cognitive impairment: presentation and long-term outcome. Neurology 2016;87:759–765.Google Scholar
Titulaer, MJ, McCracken, L, Gabilondo, I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013;12:157–165.Google Scholar
Spatola, M, Petit-Pedrol, M, Simabukuro, MM, et al. Investigations in GABAA receptor antibody-associated encephalitis. Neurology 2017;88:1012–1020.Google Scholar
Ogawa, R, Nakashima, I, Takahashi, T, et al. MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy. Neurol Neuroimmunol Neuroinflamm 2017;4:e322.Google Scholar
Armangue, T, Olive-Cirera, G, Martinez-Hernandez, E, et al. Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study. Lancet Neurol 2020;19:234–246.Google Scholar
Graus, F, Gorman, MP. Voltage-gated potassium channel antibodies: game over. Neurology 2016;86:1657–1658.Google Scholar
Dubey, D, Pittock, SJ, Kelly, CR, et al. Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. Ann Neurol 2018;83:166–177.Google Scholar
Venkatesan, A, Tunkel, AR, Bloch, KC, et al. Case definitions, diagnostic algorithms, and priorities in encephalitis: consensus statement of the international encephalitis consortium. Clin Infect Dis 2013;57:1114–1128.Google Scholar
Gaig, C, Graus, F, Compta, Y, et al. Clinical manifestations of the anti-IgLON5 disease. Neurology 2017;88:1736–1743.Google Scholar
Gresa-Arribas, N, Titulaer, MJ, Torrents, A, et al. Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: a retrospective study. Lancet Neurol 2014;13:167–177.Google Scholar
Hara, M, Martinez-Hernandez, E, Ariño, H, et al. Clinical and pathogenic significance of IgG, IgA, and IgM antibodies against the NMDA receptor. Neurology 2018;90:e1386–e1394.Google Scholar
van Sonderen, A, Thijs, RD, Coenders, EC, et al. Anti-LGI1 encephalitis: clinical syndrome and long-term follow-up. Neurology 2016;87:1449–1456.Google Scholar
Gable, MS, Sheriff, H, Dalmau, J, Tilley, DH, Glaser, CA. The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. Clin Infect Dis 2012;54:899–904.Google Scholar
Pohl, D, Hennemuth, I, von Kries, R, Hanefeld, F. Paediatric multiple sclerosis and acute disseminated encephalomyelitis in Germany: results of a nationwide survey. Eur J Pediatr 2007;166:405–412.CrossRefGoogle ScholarPubMed
Banwell, B, Kennedy, J, Sadovnick, D, et al. Incidence of acquired demyelination of the CNS in Canadian children. Neurology 2009;72:232–239.Google Scholar
Torisu, H, Kira, R, Ishizaki, Y, et al. Clinical study of childhood acute disseminated encephalomyelitis, multiple sclerosis, and acute transverse myelitis in Fukuoka Prefecture, Japan. Brain Dev 2010;32:454–462.Google Scholar
Leake, JA, Albani, S, Kao, AS, et al. Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr Infect Dis J 2004;23:756–764.Google Scholar
Reindl, M, Waters, P. Myelin oligodendrocyte glycoprotein antibodies in neurological disease. Nat Rev Neurol 2019;15:89–102.Google Scholar
Gelpi, E, Hoftberger, R, Graus, F, et al. Neuropathological criteria of anti-IgLON5-related tauopathy. Acta Neuropathol 2016;132:531–543.Google Scholar
Dalmau, J, Graus, F, Villarejo, A, et al. Clinical analysis of anti-Ma2-associated encephalitis. Brain 2004;127:1831–1844.Google Scholar
Hacohen, Y, Wright, S, Waters, P, et al. Paediatric autoimmune encephalopathies: clinical features, laboratory investigations and outcomes in patients with or without antibodies to known central nervous system autoantigens. J Neurol Neurosurg Psychiatry 2013;84:748–755.Google Scholar
Zamvil, SS, Slavin, AJ. Does MOG Ig-positive AQP4-seronegative opticospinal inflammatory disease justify a diagnosis of NMO spectrum disorder? Neurol Neuroimmunol Neuroinflamm 2015;2:e62.Google Scholar
Dubey, D, Hinson, SR, Jolliffe, EA, et al. Autoimmune GFAP astrocytopathy: prospective evaluation of 90 patients in 1 year. J Neuroimmunol 2018;321:157–163.Google Scholar

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