Abordaje clínico para su diagnóstico | 04 ABR 16

Encefalitis autoimmune

Trastorno neurológico debilitante que se desarrolla como una encefalopatía rápidamente progresiva causada por la inflamación cerebral.
Autor/a: Francesc Graus, Maarten J Titulaer, Ramani Balu, Susanne Benseler, Christian G Bien The Lancet Volume 15, No. 4, p391–404, April 2016. A clinical approach to diagnosis of autoimmune encephalitis
INDICE:  1. Página 1 | 2. Referencias

1.Venkatesan A, Tunkel AR, Bloch KC, et al, for the International Encephalitis Consortium. Case defi nitions, diagnostic algorithms, and priorities in encephalitis: consensus statement of the international encephalitis consortium. Clin Infect Dis 2013; 57: 1114–28.
2 Jmor F, Emsley HC, Fischer M, Solomon T, Lewthwaite P. The incidence of acute encephalitis syndrome in Western industrialised and tropical countries. Virol J 2008; 5: 134–46.
3 Vora NM, Holman RC, Mehal JM, Steiner CA, Blanton J, Sejvar J. Burden of encephalitis-associated hospitalizations in the United States, 1998–2010. Neurology 2014; 82:443–51.
4 Ball R, Halsey N, Braun MM, et al, for the VAERS Working Group. Development of case defi nitions for acute encephalopathy, encephalitis, and multiple sclerosis reports to the vaccine: Adverse Event Reporting System. J Clin Epidemiol 2002; 55:819–24
5 Sejvar JJ, Kohl KS, Bilynsky R, et al, for the Brighton Collaboration Encephalitis Working Group. Encephalitis, myelitis, and acute disseminated encephalomyelitis (ADEM): case defi nitions and guidelines for collection, analysis, and presentation of immunization safety data. Vaccine 2007; 25: 5771–92.
6 Britton PN, Eastwood K, Paterson B, et al, for the Australasian Society of Infectious Diseases (ASID), the Australasian College of Emergency Medicine (ACEM), the Australian and New Zealand Association of Neurologists (ANZAN), and the Public Health Association of Australia (PHAA). Consensus guidelines for the investigation and management of encephalitis in adults and children in Australia and New Zealand. Intern Med J 2015; 45: 563–76.
7 Leypoldt F, Armangue T, Dalmau J. Autoimmune encephalopathies. Ann N Y Acad Sci 2015; 1338: 94–114.
8 Alamowitch S, Graus F, Uchuya M, Rene R, Bescansa E, Delattre JY. Limbic encephalitis and small cell lung cancer. Clinical and immunological features. Brain 1997; 120: 923–28.
9 Dalmau J, Graus F, Villarejo A, et al. Clinical analysis of anti-Ma2-associated encephalitis. Brain 2004; 127: 1831–44.
10 Malter MP, Helmstaedter C, Urbach H, Vincent A, Bien CG. Antibodies to glutamic acid decarboxylase defi ne a form of limbic encephalitis. Ann Neurol 2010; 67: 470–78.
11 Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011;10: 63–74.
12 Lai M, Hughes EG, Peng X, et al. AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol 2009; 65: 424–34.
13 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.
14 Petit-Pedrol M, Armangue T, Peng X, et al. Encephalitis with refractory seizures, status epilepticus, and antibodies to the GABAA receptor: a case series, characterisation of the antigen, and analysis of the eff ects of antibodies. Lancet Neurol 2014; 13: 276–86.
15 Lancaster E, Martinez-Hernandez E, Titulaer MJ, et al. Antibodies to metabotropic glutamate receptor 5 in the Ophelia syndrome. Neurology 2011; 77: 1698–701.
16 Dale RC, Merheb V, Pillai S, et al. Antibodies to surface dopamine-2 receptor in autoimmune movement and psychiatric disorders. Brain 2012; 135: 3453–68.
17 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: 776–85.
18 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: 2734–48.
19 Boronat A, Gelfand JM, Gresa-Arribas N, et al. Encephalitis and antibodies to dipeptidyl-peptidase-like protein-6, a subunit of Kv4.2 potassium channels. Ann Neurol 2013; 73: 120–28.
20 Brilot F, Dale RC, Selter RC, et al. Antibodies to native myelin oligodendrocyte glycoprotein in children with infl amatory demyelinating central nervous system disease. Ann Neurol 2009;66: 833–42.
21 McKeon A, Lennon VA, Lotze T, et al. CNS aquaporin-4 autoimmunity in children. Neurology 2008; 71: 93–100.
22 Shahrizaila N, Yuki N. Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome. J Neurol Neurosurg Psychiatry 2013; 84: 576–83.
23 Arino H, Hoftberger R, Gresa-Arribas N, et al. Paraneoplastic neurological syndromes and glutamic acid decarboxylase antibodies. JAMA Neurol 2015; 72: 874–81.
24 Alexopoulos H, Dalakas MC. Immunology of stiff person dyndrome and other GAD-associated neurological disorders. Expert Rev Clin Immunol 2013; 9: 1043–53.
25 Carvajal-Gonzalez A, Leite MI, Waters P, et al. Glycine receptor antibodies in PERM and related syndromes: characteristics, clinical features and outcomes. Brain 2014; 137: 2178–92.
26 Demarquay G, Honnorat J. Clinical presentation of immune-mediated cerebellar ataxia. Rev Neurol (Paris) 2011;167: 408–17.
27 Zuliani L, Graus F, Giometto B, Bien C, Vincent A. Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition. J Neurol Neurosurg Psychiatry 2012;83: 638–45.
28 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: 157–65.
29 Byrne S, Walsh C, Hacohen Y, et al. Earlier treatment of NMDAR antibody encephalitis in children results in a better outcome. Neurol Neuroimmunol Neuroinfl amm 2015; 2: e130.
30 Ances BM, Vitaliani R, Taylor RA, et al. Treatment-responsive limbic encephalitis identifi ed by neuropil antibodies: MRI and PET correlates. Brain 2005; 128: 1764–77.
31 Vincent A, Buckley C, Schott JM, et al. Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 2004; 127: 701–12.
32 Krupp LB, Tardieu M, Amato MP, et al, for the International Pediatric Multiple Sclerosis Study Group. International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 defi nitions. Mult Scler 2013; 19: 1261–67.
33 Schiess N, Pardo CA. Hashimoto’s encephalopathy. Ann N  Y Acad Sci 2008; 1142: 254–65.
34 Irani SR, Pettingill P, Kleopa KA, et al. Morvan syndrome: clinical and serological observations in 29 cases. Ann Neurol 2012; 72: 241–55.
35 Bien CG, Granata T, Antozzi C, et al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. Brain 2005; 128: 454–71.
36 Armangue T, Petit-Pedrol M, Dalmau J. Autoimmune encephalitis in children. J Child Neurol 2012; 27: 1460–69.
37 Pillai SC, Hacohen Y, Tantsis E, et al. Infectious and autoantibody-associated encephalitis: clinical features and long-term outcome. Pediatrics 2015; 135: e974–84.
38 Schmitt SE, Pargeon K, Frechette ES, Hirsch LJ, Dalmau J, Friedman D. Extreme delta brush: a unique EEG pattern in adults with anti-NMDA receptor encephalitis. Neurology 2012;79: 1094–100.
39 Weil AA, Glaser CA, Amad Z, Forghani B. Patients with suspected herpes simplex encephalitis: rethinking an initial negative polymerase chain reaction result. Clin Infect Dis 2002; 34: 1154–57.
40 Solomon T, Michael BD, Smith PE, et al, for the National Encephalitis Guidelines Development and Stakeholder Groups. Management of suspected viral encephalitis in adults—association of British Neurologists and British Infection Association National Guidelines. J Infect 2012; 64: 347–73.
41 Tobin WO, Lennon VA, Komorowski L, et al. DPPX potassium channel antibody: frequency, clinical accompaniments, and outcomes in 20 patients. Neurology 2014; 83: 1797–803.
42 Irani SR, Michell AW, Lang B, et al. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol 2011; 69: 892–900.
43 Wakerley BR, Uncini A, Yuki N, for the GBS Classifi cation Group, and the GBS Classifi cation Group. Guillain-Barre and Miller Fisher syndromes—new diagnostic classifi cation. Nat Rev Neurol 2014;10: 537–44.
44 Baumgartner A, Rauer S, Mader I, Meyer PT. Cerebral FDG-PET and MRI fi ndings in autoimmune limbic encephalitis: correlation with autoantibody types. J Neurol 2013; 260: 2744–53.
45 Heine J, Pruss H, Bartsch T, Ploner CJ, Paul F, Finke C. Imaging of autoimmune encephalitis - Relevance for clinical practice and hippocampal function. Neuroscience 2015; 309: 68–83.
46 Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological fi ndings and tumour association in 50 patients. Brain 2000; 123: 1481–94.
47 Graus F, Delattre JY, Antoine JC, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 2004; 75: 1135–40.
48 Graus F, Saiz A, Lai M, et al. Neuronal surface antigen antibodies in limbic encephalitis: clinical-immunologic associations. Neurology 2008; 71: 930–36.
49 Najjar S, Pearlman D, Zagzag D, Devinsky O. Spontaneously resolving seronegative autoimmune limbic encephalitis. Cogn Behav Neurol 2011; 24: 99–105.
50 Voltz R, Gultekin SH, Rosenfeld MR, et al. A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med 1999; 340: 1788–95.
51 Hoftberger R, Titulaer MJ, Sabater L, et al. Encephalitis and GABAB receptor antibodies: novel fi ndings in a new case series of 20 patients. Neurology 2013; 81: 1500–06.
52 Hoftberger R, van Sonderen A, Leypoldt F, et al. Encephalitis and AMPA receptor antibodies: novel fi ndings in a case series of 22 patients. Neurology 2015; 84: 2403–12.
53 Malter MP, Frisch C, Schoene-Bake JC, et al. Outcome of limbic encephalitis with VGKC-complex antibodies: relation to antigenic specifi city. J Neurol 2014; 261: 1695–705.
54 Jarius S, Hoff mann L, Clover L, Vincent A, Voltz R. CSF fi ndings in patients with voltage gated potassium channel antibody associated limbic encephalitis. J Neurol Sci 2008; 268: 74–77.
55 Athauda D, Delamont RS, Pablo-Fernandez ED. High grade glioma mimicking voltage gated potassium channel complex associated antibody limbic encephalitis. Case Rep Neurol Med 2014; 2014: 458790.
56 Chevret L, Husson B, Nguefack S, Nehlig A, Bouilleret V. Prolonged refractory status epilepticus with early and persistent restricted hippocampal signal MRI abnormality. J Neurol 2008; 255: 112–16.
57 Chow FC, Glaser CA, Sheriff H, et al. Use of clinical and neuroimaging characteristics to distinguish temporal lobe herpes simplex encephalitis from its mimics. lin Infect Dis 2015;
60: 1377–83.
58 Seeley WW, Marty FM, Holmes TM, et al. Post-transplant acute limbic encephalitis: clinical features and relationship to HHV6. Neurology 2007; 69: 156–65.
59 Renard D, Nerrant E, Lechiche C. DWI and FLAIR imaging in herpes simplex encephalitis: a comparative and topographical analysis. J Neurol 2015; 262: 2101–05.
60 Jeff ery OJ, Lennon VA, Pittock SJ, Gregory JK, Britton JW, McKeon A. GABAB receptor autoantibody frequency in service serologic evaluation. Neurology 2013; 81: 882–87.
61 Wingerchuk DM. The clinical course of acute disseminated encephalomyelitis. Neurol Res 2006; 28: 341–47.
62 Wingerchuk DM. Postinfectious encephalomyelitis. Curr Neurol Neurosci Rep 2003; 3: 256–64.
63 Karussis D, Petrou P. The spectrum of post-vaccination inflamatory CNS demyelinating syndromes. Autoimmun Rev 2014; 13: 215–24.
64 Wingerchuk DM, Weinshenker BG. Acute disseminated encephalomyelitis, transverse myelitis, and neuromyelitis optica. Continuum (Minneap Minn) 2013; 19: 944–67.
65 Kesselring J, Miller DH, Robb SA, et al. Acute disseminated encephalomyelitis. MRI fi ndings and the distinction from multiple sclerosis. Brain 1990; 113: 291–302.
66 Baumann M, Sahin K, Lechner C, et al. Clinical and neuroradiological diff erences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein. J Neurol Neurosurg Psychiatry 2015; 86: 265–72.
67 Huppke P, Rostasy K, Karenfort M, et al. Acute disseminated encephalomyelitis followed by recurrent or monophasic optic neuritis in pediatric patients. Mult Scler 2013; 19: 941–46.
68 Reindl M, Di Pauli F, Rostasy K, Berger T. The spectrum of MOG autoantibody-associated demyelinating diseases. Nat Rev Neurol 2013; 9: 455–61.
69 Kleff ner I, Duning T, Lohmann H, et al. A brief review of Susac syndrome. J Neurol Sci 2012; 322: 35–40.
70 Dorr J, Krautwald S, Wildemann B, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol 2013;9: 307–16.
71 Moscato EH, Peng X, Jain A, Parsons TD, Dalmau J, Balice-Gordon RJ. Acute mechanisms underlying antibody eff ects in anti-N-methyl-D-aspartate receptor encephalitis. Ann Neurol 2014;76: 108–19.
72 Planaguma J, Leypoldt F, Mannara F, et al. Human N-methyl D-aspartate receptor antibodies alter memory and behaviour in mice. Brain 2015; 138: 94–109.
73 Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the eff ects of antibodies. Lancet Neurol 2008; 7: 1091–98.
74 Viaccoz A, Desestret V, Ducray F, et al. Clinical specifi cities of adult male patients with NMDA receptor antibodies encephalitis. Neurology 2014; 82: 556–63.
75 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: 167–77.
76 Zandi MS, Paterson RW, Ellul MA, et al. Clinical relevance of serum antibodies to extracellular N-methyl-D-aspartate receptor epitopes. J Neurol Neurosurg Psychiatry 2015; 86: 708–13.
77 Wang R, Guan HZ, Ren HT, Wang W, Hong Z, Zhou D. CSF fi ndings in patients with anti-N-methyl-D-aspartate receptor-encephalitis. Seizure 2015; 29: 137–42.
78 Pruss H, Finke C, Holtje M, et al. N-methyl-D-aspartate receptor antibodies in herpes simplex encephalitis. Ann Neurol 2012; 72: 902–11.
79 Armangue T, Leypoldt F, Malaga I, et al. Herpes simplex virus encephalitis is a trigger of brain autoimmunity. Ann Neurol 2014;75: 317–23.
80 Hacohen Y, Deiva K, Pettingill P, et al. N-methyl-D-aspartate receptor antibodies in post-herpes simplex virus encephalitis neurological relapse. Mov Disord 2014; 29: 90–96.
81 Armangue T, Moris G, Cantarin-Extremera V, et al, for the Spanish Prospective Multicentric Study of Autoimmunity in Herpes Simplex Encephalitis. Autoimmune post-herpes simplex encephalitis of adults and teenagers. Neurology 2015; 85: 1736–43.
82 Mohammad SS, Sinclair K, Pillai S, et al. Herpes simplex encephalitis relapse with chorea is associated with autoantibodies to N-Methyl-D-aspartate receptor or dopamine-2 receptor. Mov Disord 2014; 29: 117–22.
83 Koga M, Kusunoki S, Kaida K, et al. Nationwide survey of patients in Japan with Bickerstaff brainstem encephalitis: epidemiological and clinical characteristics. J Neurol Neurosurg Psychiatry 2012;83: 1210–15.
84 Odaka M, Yuki N, Yamada M, et al. Bickerstaff ’s brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barre syndrome. Brain 2003; 126: 2279–90.
85 Merwick A, Dalmau J, Delanty N. Insights into antibody-associated encephalitis—Bickerstaff ’s 1950’s papers revisited. J Neurol Sci 2013; 334: 167–68.
86 Moragas M, Martinez-Yelamos S, Majos C, Fernandez-Viladrich P, Rubio F, Arbizu T. Rhombencephalitis: a series of 97 patients. Medicine (Baltimore) 2011; 90: 256–61.
87 Pittock SJ, Debruyne J, Krecke KN, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain 2010; 133: 2626–34.
88 Wang SM, Liu CC. Enterovirus 71: epidemiology, pathogenesis and management. Expert Rev Anti Infect Ther 2009; 7: 735–42.
89 Gleichman AJ, Spruce LA, Dalmau J, Seeholzer SH, Lynch DR. Anti-NMDA receptor encephalitis antibody binding is dependent on amino acid identity of a small region within the GluN1 amino terminal domain. J Neurosci 2012; 32: 11082–94.
90 Takahashi Y, Mori H, Mishina M, et al. Autoantibodies and cell-mediated autoimmunity to NMDA-type GluRepsilon2 in patients with Rasmussen’s encephalitis and chronic progressive epilepsia partialis continua. Epilepsia 2005; 46 (suppl 5): 152–58.
91 Huda S, Wong SH, Pettingill P, O’Connell D, Vincent A, Steiger M. An 11-year retrospective experience of antibodies against the voltage-gated potassium channel (VGKC) complex from a tertiary neurological centre. J Neurol 2015; 262: 418–24.
92 Paterson RW, Zandi MS, Armstrong R, Vincent A, Schott JM. Clinical relevance of positive voltage-gated potassium channel (VGKC)-complex antibodies: experience from a tertiary referral centre. J Neurol Neurosurg Psychiatry 2014; 85: 625–30.
93 Klein CJ, Lennon VA, Aston PA, et al. Insights from LGI1 and CASPR2 potassium channel complex autoantibody subtyping. JAMA Neurol 2013; 70: 229–34.
94 Dahm L, Ott C, Steiner J, et al. Seroprevalence of autoantibodies against brain antigens in health and disease. Ann Neurol 2014;76: 82–94.
95 Armangue T, Santamaria J, Dalmau J. When a serum test overrides the clinical assessment. Neurology 2015; 84: 1379–81.
96 Titulaer MJ, Hoftberger R, Iizuka T, et al. Overlapping demyelinating syndromes and anti-N-methyl-D-aspartate receptor encephalitis. Ann Neurol 2014; 75: 411–28.
97 Jarius S, Paul F, Franciotta D, et al. Cerebrospinal fl uid fi ndings in aquaporin-4 antibody positive neuromyelitis optica: results from 211 lumbar punctures. J Neurol Sci 2011; 306: 82–90.
98 Meinck HM, Faber L, Morgenthaler N, et al. Antibodies against glutamic acid decarboxylase: prevalence in neurological diseases. J Neurol Neurosurg Psychiatry 2001; 71: 100–03.
99 Saiz A, Blanco Y, Sabater L, et al. Spectrum of neurological syndromes associated with glutamic acid decarboxylase antibodies: diagnostic clues for this association. Brain 2008; 131: 2553–63.
100 Hollowell JG, Staehling NW, Flanders WD, et al. Serum TSH, T(4), and thyroid antibodies in the United States population (1988 to 1994): National Health and Nutrition Examination Survey (NHANES III). J Clin Endocrinol Metab 2002; 87: 489–99.
101 Castillo P, Woodruff B, Caselli R, et al. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol 2006; 63: 197–202.
102 Tardieu M, Deiva K. Rare infl ammatory diseases of the white matter and mimics of multiple sclerosis and related disorders. Neuropediatrics 2013; 44: 302–08.
103 Brain L, Jellinek EH, Ball K. Hashimoto’s disease and encephalopathy. Lancet 1966; 2: 512–14. 104 Chong JY, Rowland LP, Utiger RD. Hashimoto encephalopathy: syndrome or myth? Arch Neurol 2003; 60: 164–71.
105 Yoneda M, Fujii A, Ito A, Yokoyama H, Nakagawa H, Kuriyama M. High prevalence of serum autoantibodies against the amino terminal of alpha-enolase in Hashimoto’s encephalopathy. J Neuroimmunol 2007; 185: 195–200.
106 Servettaz A, Guilpain P, Tamas N, Kaveri SV, Camoin L, Mouthon L. Natural anti-endothelial cell antibodies. Autoimmun Rev 2008; 7: 426–30.
107 Pettingill P, Kramer HB, Coebergh JA, et al. Antibodies to GABAA receptor α1 and γ2 subunits: clinical and serologic characterization. Neurology 2015; 84: 1233–41.
108 Hajj-Ali RA, Singhal AB, Benseler S, Molloy E, Calabrese LH. Primary angiitis of the CNS. Lancet Neurol 2011; 10: 561–72.
109 Nabbout R. FIRES and IHHE: delineation of the syndromes. Epilepsia 2013; 54 (suppl 6): 54–56



Usted debe ingresar al sitio con su cuenta de usuario IntraMed para ver los comentarios de sus colegas o para expresar su opinión. Si ya tiene una cuenta IntraMed o desea registrase, ingrese aquí

Términos y condiciones de uso | Todos los derechos reservados | Copyright 1997-2021