Resumen
INTRODUCCIÓN:La encefalitis autoinmune es causada por mecanismos inmunes antineuronales, su presentación clínica es heterogénea, los criterios clínicos y paraclínicos disponibles orientan el abordaje, sin embargo, el reto ocurre cuando no hay autoanticuerpos detectables en suero o líquido cefalorraquídeo (LCR).
METODOLOGÍA:Reportamos cuatro casos destacando la variabilidad de las manifestaciones clínicas, que ante la ausencia de anticuerpos (negativos finalmente en tres de los casos) fueron tratados con inmunoterapia con buena respuesta.
CONCLUSIÓN:En sitios donde no se dispone de medición de anticuerpos de manera expedita, o a pesar de ser estos negativos, ante la sospecha clínica, apoyado de estudios de LCR, resonancia magnética nuclear y registro electroencefalográfico, se sugiere iniciar inmunoterapia temprana, usualmente dando lugar a reversibilidad del trastorno neurológico.
Citas
Granerod J, Ambrose HE, Davies NWS, Clewley JP, Walsh AL, Morgan D, et al. Causes of encephalitis and differences in their clinical presentations in England: A multicentre, population-based prospective study. Lancet Infect Dis. 2010;10(12):835-44.
Gable M, Glaser C. Anti-N-Methyl-D-Aspartate receptor encephalitis appearing as a new-onset psychosis: Disease course in children and adolescents within the California Encephalitis Project. Pediatr Neurol. 2017;72:25-30.
Affan M, Madani S, Osman G, Alsrouji O, Schultz L, Cerghet M. Clinical presentation and treatment of autoimmune encephalitis: single center cohort study of 47 cases. Neurology. 2018;90(15 supplement):P5.387.
Dubey D, Pittock SJ, Kelly CR et al. Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. Ann Neurol. 2018;83(1):166-177.
Zhao J, Guan H, Zhang J, Wang C, Xu X, Zhang Y, et al. Coexistence of autoimmune encephalitis and other systemic autoimmune diseases. Front Neurol. 2019;10(1142).
Alexopoulos H, Dalakas MC. The immunobiology of autoimmune encephalitides. J Autoimmun. 2019 Nov;104:102339.
Giordano A, Fazio R, Gelibter S, Minicucci F, Vabanesi M, Anzalone N, et al. Diagnosing autoimmune encephalitis in a real-world single-centre setting. J Neurol. 2019;1-12.
Netravathi M. Seronegative autoimmune encephalitis: A diagnostic and therapeutic dilemma. Ann Indian Acad Neurol. 2019;22(4):369-370.
Tsai M, Lin C, Tsai K, Lin M, Ho C, Lu Y, et al. S100A6 promotes B lymphocyte penetration through the blood - brain barrier in autoimmune encephalitis. Front Genet. 2019;10(November):1-9.
Hansen N, Schwing K, Onder D, Widman G, Leelaarporn P, Prusseit I, et al. Low CSF CD4 / CD8 + T-cell proportions are associated with blood - CSF barrier dysfunction in limbic encephalitis. Epilepsy Behav. 2020;102:1-6.
Wesselingh R, Butzkueven H, Buzzard K, Tarlinton D. Innate immunity in the central nervous system : A missing piece of the autoimmune encephalitis puzzle ? Front Immunol. 2019 Sep 10;10:2066.
Behrman S, Lennox B. Autoimmune encephalitis in the elderly : who to test and what to test for. Evid Based Ment Health. 2019;22:172-6.
Herken J, Prùss H. Red flags: Clinical signs for identifying autoimmune encephalitis in psychiatric patients. Front Psychiatry. 2017;8(FEB):1-9.
Sarkis RA, Nehme R, Chemali ZN. Neuropsychiatric and seizure outcomes in nonparaneoplastic autoimmune limbic encephalitis. Epilepsy Behav. 2014;39:21-5.
Hoftberger R, Titulaer MJ, Sabater L, Dome B, Rozsas A, Hegedus B, et al. Encephalitis and GABAB receptor antibodies: novel findings in a new case series of 20 patients. Neurology. 2013;81(17):1500-6.
Kiani R, Lawden M, Eames P, Critchley P, Bhaumik S, Odedra S, et al. Anti-NMDA-receptor encephalitis presenting with catatonia and neuroleptic malignant syndrome in patients with intellectual disability and autism. BJPsych Bull. 2015;39(1):32-5.
Lejuste F, Thomas L, Picard G, Desestret V, Ducray F, Rogemond V, et al. Neuroleptic intolerance in patients with anti-NMDAR encephalitis. Neurol - Neuroimmunol Neuroinflammation. 2016;3(5):e280.
Janas-kozik M, Krzystanek M, Cichon L, Jelonek I, Siwiec A, Rybakowski JK, et al. Case Report An adolescent case of limbic encephalitis with anti leucine- rich glioma inactivated 1 antibodies Case Report. Neuropsychiatry (London). 2017;7:179-82.
Vincent A, Kneen R. VGKC-complex antibody mediated encephalitis presenting with psychiatric features and neuro-leptic malignant syndrome - further expanding the phenotype Anand. Dev Child Neurol. 2012;54:575-6.
Soeder BM, Gleissner U, Urbach H, Clusmann H, Elger CE, Vincent A, et al. Causes, presentation and outcome of lesional adult onset mediotemporal lobe epilepsy. J Neurol Neurosurg Psychiatry. 2009;80(8):894-9.
Wright S, Vincent A. Europe PMC Funders Group Progress in autoimmune epileptic encephalitis. Curr Opin Neurol. 2016;29(2):151-7.
Lancaster E. The diagnosis and treatment of autoimmune encephalitis. J Clin Neurol. 2016;12(1):1-13.
Goodfellow JA, Mackay GA. Autoimmune encephalitis. J R Coll Physicians Edinb. 2019;49(4):287-294.
Ren C, Nai Y, Lv W, Liu H, Chen Q, Sun Z, et al. Focus on autonomic dysfunctions in anti-NMDAR encephalitis : a case report. Eur Rev Med Pharmacol Sci. 2019;23:10970-5.
Serafini A, Lukas R V., VanHaerents S, Warnke P, Tao JX, Rose S, et al. Paraneoplastic epilepsy. Epilepsy Behav. 2016;61:51-8.
Bahat E, Akman S, Karpuzoglu G, Aktan S, Ucar T, Arslan AG, et al. Visceral Kaposi's sarcoma with intracranial metastasis: a rare complication of renal transplantation. Pediatr Transplant. 2002;6(6):505-8.
Inhoff O, Doerries K, Doerries R, Scharf J, Groden C, Goerdt S, et al. Disseminated cutaneous Kaposi sarcoma and progressive multifocal leukoencephalopathy in a patient with idiopathic CD4+ T lymphocytopenia. Arch Dermatol. 2007;143(5):673-5.
Geraminejad P, Memar O, Aronson I, Rady PL, Hengge U, Tyring SK. Kaposi's sarcoma and other manifestations of human herpesvirus 8. J Am Acad Dermatol. 2002;47(5):641-55.
Mantero V, Mascolo M, Bandettini di Poggio M, Caponnetto C, Pardini M. Myasthenia gravis developing in an HIV-negative patient with Kaposi's sarcoma. Neurol Sci. 2013 Jul;34(7):1249-50.
Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15(4):391-404.
Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, 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(2):157-65.
Wandinger KP, Saschenbrecker S, Stoecker W, Dalmau J. Anti-NMDA-receptor encephalitis: A severe, multistage, treatable disorder presenting with psychosis. J Neuroimmunol. 2011;231(1-2):86-91.
Lee SK, Lee S-T. The laboratory diagnosis of autoimmune encephalitis. J Epilepsy Res. 2016;6(2):45-50.
Wang R, Guan HZ, Ren HT, Wang W, Hong Z, Zhou D. CSF findings in patients with anti-N-methyl-d-aspartate receptor-encephalitis. Seizure. 2015;29:137-42.
Moise A, Karakis I, Herlopian A, Dhakar M, Hirsch LJ, Cotsonis G, et al. Continuous EEG findings in autoimmune encephalitis. J Clin Neurophysiol. 2019 Dec 2. doi: 10.1097/WNP.0000000000000654.
Foundation IM, Gemelli P, Antonio OS, Euganea A, Martino IPS, Clinic N, et al. Subgroup comparison according to clinical phenotype and serostatus in autoimmune encephalitis: a multicenter retrospective study. Eur J Neurol. 2019;Dec 8. doi: 10.1111/ene.14139.
Teixeira S, Santos JC, Real MV, Santos F. Autoimmune encephalitis : the clinical evolution as a key to the diagnosis. BMJ Case Rep. 2019;12:11-3.
Xu X, Lu Q, Huang Y, Fan S, Zhou L, Yuan J, et al. Anti-NMDAR encephalitis. Neurol Neuroimmunol Neuroinflam. 2020;7(1):1-9.
Guasp M, Ariño H, Dalmau J. Encefalitis autoinmunes. Rev Neurol. 2018;66(Supl 2):1-6.
Joubert B, Dalmau J. The role of infections in autoimmune encephalitides. Rev Neurol (Paris). 2019;175(7-8):420-6.
Esta obra está bajo una licencia internacional Creative Commons Atribución-NoComercial-SinDerivadas 4.0.