Encefalitis límbica asociada a secreción inadecuada de hormona antidiurética
Reporte de un caso y revisión de la literatura
Palabras clave:
ENCEFALITIS LÍMBICA, HIPONATREMIA, SÍNDROME DE SECRECIÓN INADECUADA DE ADH, DEMENCIA
Resumen
La encefalitis límbica (EL) autoinmune es una afección neurológica infrecuente de curso subagudo con manifestaciones neuropsicológicas. Actualmente el tratamiento inmunoterápico agudo o de mantenimiento es dirigido según el anticuerpo neural acompañante y la presencia o ausencia de cáncer.
Presentamos el caso de una mujer de 52 años con hipotiroidismo autoinmune, síndrome de secreción inadecuada de hormona antidiurética (SIADH) e hiponatremia (hipoNa) persistente, con evolución progresiva de perdida de la memoria y crisis distónicas faciobraquiales (DFBC) a quien se le realiza un diagnóstico oportuno de encefalitis límbica.
Recibió tratamiento intravenoso combinado en base a corticoides e inmunoglobulina con buena respuesta y morbilidad mínima neuropsicológica.
El reconocimiento de esta patología permite un diagnóstico y tratamiento temprano, imprescindible para mejorar el pronóstico de estos pacientes.
Citas
1) Granerod J, Ambrose H, Davies N, Clewley J, Walsh A, Morgan D, et al. Causes of encephalitis and differences in their clinical presenta-tions in England: a multicentre, population-based prospective study. Lancet Infect Dis 2010; 10(12):835-44. doi: 10.1016/S1473-3099(10)70222-X.
2) Ramanathan S, Mohammad S, Brilot F, Dale R. Autoimmune en-cephalitis: recent updates and emerging challenges. J Clin Neurosci 2014; 21(5):722-30. doi: 10.1016/j.jocn.2013.07.017.
3) Kumar R. Understanding and managing acute encephalitis. F1000Res 2020; 9:F1000 Faculty Rev-60. doi: 10.12688/f1000research.20634.1.
4) da Rocha A, Nunes R, Maia AJr, do Amaral L. Recognizing autoim-mune-mediated encephalitis in the differential diagnosis of limbic disorders. AJNR Am J Neuroradiol 2015; 36(12):2196-205. doi: 10.3174/ajnr.A4408.
5) Bien C, Vincent A, Barnett M, Becker A, Blümcke I, Graus F, et al. Immunopathology of autoantibody-associated encephalitides: clues for pathogenesis. Brain 2012; 135(Pt 5):1622-38. doi: 10.1093/brain/aws082.
6) Banks S, Sechi E, Flanagan E. Autoimmune encephalopathies pre-senting as dementia of subacute onset and rapid progression. Ther Adv Neurol Disord 2021; 14:1756286421998906. doi: 10.1177/1756286421998906.
7) Dalmau J, Graus F. Antibody-mediated encephalitis. N Engl J Med 2018; 378(9):840-51. doi: 10.1056/NEJMra1708712.
8) Jammoul A, Li Y, Rae-Grant A. Autoantibody-mediated encephalitis: not just paraneoplastic, not just limbic, and not untreatable. Cleve Clin J Med 2016; 83(1):43-53. doi: 10.3949/ccjm.83a.14112.
9) Graus F, Titulaer M, Balu R, Benseler S, Bien C, Cellucci T, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016; 15(4):391-404. doi: 10.1016/S1474-4422(15)00401-9.
10) Ortega Suero G, Sola-Valls N, Escudero D, Saiz A, Graus F. Anti-Ma and anti-Ma2-associated paraneoplastic neurological syn-dromes. Neurologia (Engl Ed) 2018; 33(1):18-27. doi: 10.1016/j.nrl.2016.05.010.
11) Soler B, Godoy J, Mellado, P. Encefalitis límbica por anticuer-pos anticanales de potasio dependientes de voltaje: caso clínico. Rev Méd Chile 2009; 137(5):675-9. doi: 10.4067/S0034-98872009000500012.
12) Corsellis J, Goldberg G, Norton A. Limbic encephalitis and its association with carcinoma. Brain 1968; 91(3):481-96. doi: 10.1093/brain/91.3.481.
13) Gultekin S, Rosenfeld M, Voltz R, Eichen J, Posner J, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immu-nological findings and tumour association in 50 patients. Brain 2000; 123(Pt 7):1481-94. doi: 10.1093/brain/123.7.1481.
14) Dalmau J, Lancaster E, Martínez-Hernández E, Rosenfeld M, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011; 10(1):63-74. doi: 10.1016/S1474-4422(10)70253-2.
15) Vincent A, Buckley C, Schott J, Baker I, Dewar B, Detert N, et al. Potassium channel antibody-associated encephalopathy: a po-tentially immunotherapy-responsive form of limbic encephalitis. Brain 2004; 127(Pt 3):701-12. doi: 10.1093/brain/awh077.
16) Shen K, Xu Y, Guan H, Zhong W, Chen M, Zhao J, et al. Paraneoplastic limbic encephalitis associated with lung cancer. Sci Rep 2018; 8(1):6792. doi: 10.1038/s41598-018-25294-y.
17) Moise A, Karakis I, Herlopian A, Dhakar M, Hirsch L, Cotsonis G, et al. Continuous EEG findings in autoimmune encephalitis. J Clin Neurophysiol 2021; 38(2):124-9. doi: 10.1097/WNP.0000000000000654.
18) van Sonderen A, Thijs R, Coenders E, Jiskoot L, Sanchez E, de Bruijn M, et al. Anti-LGI1 encephalitis: Clinical syndrome and long-term follow-up. Neurology 2016; 87(14):1449-56. doi: 10.1212/WNL.0000000000003173.
19) Qiao J, Zhao X, Wang S, Li A, Wang Z, Cao C, et al. Functional and structural brain alterations in encephalitis with LGI1 antibodies. Front Neurosci 2020; 14:304. doi: 10.3389/fnins.2020.00304.
20) Renjen P, Chaudhari D. A review of autoimmune encephalitis. Int J Sci Res 2017; 6(12):286-8.
21) Degnan A, Levy L. Neuroimaging of rapidly progressive de-mentias, part 1: neurodegenerative etiologies. AJNR Am J Neuro-radiol 2014; 35(3):418-23. doi: 10.3174/ajnr.A3454.
22) Wang S, Zhao Y, Wang Q, Guo B, Liu Y, Yan C. Pearls & Oy-sters: limbic encephalitis associated with positive anti-LGI1 and antithyroid antibodies. Neurology 2016; 86(2):e16-8. doi: 10.1212/WNL.0000000000002259.
23) Tamagno G, Celik Y, Simó R, Dihné M, Kimura K, Gelosa G, et al. Encephalopathy associated with autoimmune thyroid disease in patients with Graves’ disease: clinical manifestations, follow-up, and outcomes. BMC Neurol 2010; 10:27. doi: 10.1186/1471-2377-10-27.
24) Luster J, Barasa A, Hoffman W. Rapidly progressive dementia with recurrent seizures and hyponatremia: a case of LGI1 limbic encephalitis. Neurology 2022; 99(23 Suppl 2):S45. Doi: 10.1212/01.wnl.0000903360.38972.63.
25) Wu H, Mei F, Liu L, Zhang L, Hao H, Zhang S. Case Re-port/Case Series: rare case of anti-LGI1 limbic encephalitis with rapidly progressive dementia, psychiatric symptoms, and frequently seizures: a case report. Medicine (Baltimore) 2021; 100(29):e26654. doi: 10.1097/MD.0000000000026654.
26) Li X, Yuan J, Liu L, Hu W. Antibody-LGI 1 autoimmune en-cephalitis manifesting as rapidly progressive dementia and hypo-natremia: a case report and literature review. BMC Neurol 2019; 19(1):19. doi: 10.1186/s12883-019-1251-4.
27) Griffith S, Malpas C, Alpitsis R, O’Brien T, Monif M. The neu-ropsychological spectrum of anti-LGI1 antibody mediated autoim-mune encephalitis. J Neuroimmunol 2020; 345:577271. doi: 10.1016/j.jneuroim.2020.577271.
28) Younger D. Autoimmune encephalitides. World J Neurosci 2017; 7(3):327-61. doi:10.4236/wjns.2017.73027.
29) Chandran, S. Anti LGI1 encephalitis–a retrospective analysis of clinical features and treatment response. Neurology 2021; 96(Suppl 15):e2716.
30) Lai M, Huijbers M, Lancaster E, Graus F, Bataller L, Balice-Gordon R, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case se-ries. Lancet Neurol 2010; 9(8):776-85. doi: 10.1016/S1474-4422(10)70137-X.
31) Head K, Gong S, Joseph S, Wang C, Burkhardt T, Rossi M, et al. Defining the expression pattern of the LGI1 gene in BAC trans-genic mice. Mamm Genome 2007; 18(5):328-37. doi: 10.1007/s00335-007-9024-6.
32) Bien C. Management of autoimmune encephalitis. Curr Opin Neurol 2021; 34(2):166-71. doi: 10.1097/WCO.0000000000000909.
33) Flanagan E, McKeon A, Lennon V, Boeve B, Trenerry M, Tan K, et al. Autoimmune dementia: clinical course and predictors of im-munotherapy response. Mayo Clin Proc 2010; 85(10):881-97. doi: 10.4065/mcp.2010.0326.
34) Lee W, Lee S, Byun J, Sunwoo J, Kim T, Lim J, et al. Rituximab treatment for autoimmune limbic encephalitis in an institutional co-hort. Neurology 2016; 86(18):1683-91. doi: 10.1212/WNL.0000000000002635.
35) Hermetter C, Fazekas F, Hochmeister S. Systematic review: syndromes, early diagnosis, and treatment in autoimmune enceph-alitis. Front Neurol 2018; 9:706. doi: 10.3389/fneur.2018.00706.
36) Deuschl C, Rüber T, Ernst L, Fendler W, Kirchner J, Mönning-hoff C, et al. 18F-FDG-PET/MRI in the diagnostic work-up of limbic encephalitis. PLoS One 2020; 15(1):e0227906. doi: 10.1371/journal.pone.0227906.
37) Gadoth A, Nisnboym M, Alcalay Y, Weinstein T. LGI1 enceph-alitis associated hyponatremia, is it all in the brain? Neurology 2021; 96(Suppl 15):e2256.
38) von Rhein B, Wagner J, Widman G, Malter M, Elger C, Helm-staedter C. Suspected antibody negative autoimmune limbic en-cephalitis: outcome of immunotherapy. Acta Neurol Scand 2017; 135(1):134-41. doi: 10.1111/ane.12575.
2) Ramanathan S, Mohammad S, Brilot F, Dale R. Autoimmune en-cephalitis: recent updates and emerging challenges. J Clin Neurosci 2014; 21(5):722-30. doi: 10.1016/j.jocn.2013.07.017.
3) Kumar R. Understanding and managing acute encephalitis. F1000Res 2020; 9:F1000 Faculty Rev-60. doi: 10.12688/f1000research.20634.1.
4) da Rocha A, Nunes R, Maia AJr, do Amaral L. Recognizing autoim-mune-mediated encephalitis in the differential diagnosis of limbic disorders. AJNR Am J Neuroradiol 2015; 36(12):2196-205. doi: 10.3174/ajnr.A4408.
5) Bien C, Vincent A, Barnett M, Becker A, Blümcke I, Graus F, et al. Immunopathology of autoantibody-associated encephalitides: clues for pathogenesis. Brain 2012; 135(Pt 5):1622-38. doi: 10.1093/brain/aws082.
6) Banks S, Sechi E, Flanagan E. Autoimmune encephalopathies pre-senting as dementia of subacute onset and rapid progression. Ther Adv Neurol Disord 2021; 14:1756286421998906. doi: 10.1177/1756286421998906.
7) Dalmau J, Graus F. Antibody-mediated encephalitis. N Engl J Med 2018; 378(9):840-51. doi: 10.1056/NEJMra1708712.
8) Jammoul A, Li Y, Rae-Grant A. Autoantibody-mediated encephalitis: not just paraneoplastic, not just limbic, and not untreatable. Cleve Clin J Med 2016; 83(1):43-53. doi: 10.3949/ccjm.83a.14112.
9) Graus F, Titulaer M, Balu R, Benseler S, Bien C, Cellucci T, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016; 15(4):391-404. doi: 10.1016/S1474-4422(15)00401-9.
10) Ortega Suero G, Sola-Valls N, Escudero D, Saiz A, Graus F. Anti-Ma and anti-Ma2-associated paraneoplastic neurological syn-dromes. Neurologia (Engl Ed) 2018; 33(1):18-27. doi: 10.1016/j.nrl.2016.05.010.
11) Soler B, Godoy J, Mellado, P. Encefalitis límbica por anticuer-pos anticanales de potasio dependientes de voltaje: caso clínico. Rev Méd Chile 2009; 137(5):675-9. doi: 10.4067/S0034-98872009000500012.
12) Corsellis J, Goldberg G, Norton A. Limbic encephalitis and its association with carcinoma. Brain 1968; 91(3):481-96. doi: 10.1093/brain/91.3.481.
13) Gultekin S, Rosenfeld M, Voltz R, Eichen J, Posner J, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immu-nological findings and tumour association in 50 patients. Brain 2000; 123(Pt 7):1481-94. doi: 10.1093/brain/123.7.1481.
14) Dalmau J, Lancaster E, Martínez-Hernández E, Rosenfeld M, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011; 10(1):63-74. doi: 10.1016/S1474-4422(10)70253-2.
15) Vincent A, Buckley C, Schott J, Baker I, Dewar B, Detert N, et al. Potassium channel antibody-associated encephalopathy: a po-tentially immunotherapy-responsive form of limbic encephalitis. Brain 2004; 127(Pt 3):701-12. doi: 10.1093/brain/awh077.
16) Shen K, Xu Y, Guan H, Zhong W, Chen M, Zhao J, et al. Paraneoplastic limbic encephalitis associated with lung cancer. Sci Rep 2018; 8(1):6792. doi: 10.1038/s41598-018-25294-y.
17) Moise A, Karakis I, Herlopian A, Dhakar M, Hirsch L, Cotsonis G, et al. Continuous EEG findings in autoimmune encephalitis. J Clin Neurophysiol 2021; 38(2):124-9. doi: 10.1097/WNP.0000000000000654.
18) van Sonderen A, Thijs R, Coenders E, Jiskoot L, Sanchez E, de Bruijn M, et al. Anti-LGI1 encephalitis: Clinical syndrome and long-term follow-up. Neurology 2016; 87(14):1449-56. doi: 10.1212/WNL.0000000000003173.
19) Qiao J, Zhao X, Wang S, Li A, Wang Z, Cao C, et al. Functional and structural brain alterations in encephalitis with LGI1 antibodies. Front Neurosci 2020; 14:304. doi: 10.3389/fnins.2020.00304.
20) Renjen P, Chaudhari D. A review of autoimmune encephalitis. Int J Sci Res 2017; 6(12):286-8.
21) Degnan A, Levy L. Neuroimaging of rapidly progressive de-mentias, part 1: neurodegenerative etiologies. AJNR Am J Neuro-radiol 2014; 35(3):418-23. doi: 10.3174/ajnr.A3454.
22) Wang S, Zhao Y, Wang Q, Guo B, Liu Y, Yan C. Pearls & Oy-sters: limbic encephalitis associated with positive anti-LGI1 and antithyroid antibodies. Neurology 2016; 86(2):e16-8. doi: 10.1212/WNL.0000000000002259.
23) Tamagno G, Celik Y, Simó R, Dihné M, Kimura K, Gelosa G, et al. Encephalopathy associated with autoimmune thyroid disease in patients with Graves’ disease: clinical manifestations, follow-up, and outcomes. BMC Neurol 2010; 10:27. doi: 10.1186/1471-2377-10-27.
24) Luster J, Barasa A, Hoffman W. Rapidly progressive dementia with recurrent seizures and hyponatremia: a case of LGI1 limbic encephalitis. Neurology 2022; 99(23 Suppl 2):S45. Doi: 10.1212/01.wnl.0000903360.38972.63.
25) Wu H, Mei F, Liu L, Zhang L, Hao H, Zhang S. Case Re-port/Case Series: rare case of anti-LGI1 limbic encephalitis with rapidly progressive dementia, psychiatric symptoms, and frequently seizures: a case report. Medicine (Baltimore) 2021; 100(29):e26654. doi: 10.1097/MD.0000000000026654.
26) Li X, Yuan J, Liu L, Hu W. Antibody-LGI 1 autoimmune en-cephalitis manifesting as rapidly progressive dementia and hypo-natremia: a case report and literature review. BMC Neurol 2019; 19(1):19. doi: 10.1186/s12883-019-1251-4.
27) Griffith S, Malpas C, Alpitsis R, O’Brien T, Monif M. The neu-ropsychological spectrum of anti-LGI1 antibody mediated autoim-mune encephalitis. J Neuroimmunol 2020; 345:577271. doi: 10.1016/j.jneuroim.2020.577271.
28) Younger D. Autoimmune encephalitides. World J Neurosci 2017; 7(3):327-61. doi:10.4236/wjns.2017.73027.
29) Chandran, S. Anti LGI1 encephalitis–a retrospective analysis of clinical features and treatment response. Neurology 2021; 96(Suppl 15):e2716.
30) Lai M, Huijbers M, Lancaster E, Graus F, Bataller L, Balice-Gordon R, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case se-ries. Lancet Neurol 2010; 9(8):776-85. doi: 10.1016/S1474-4422(10)70137-X.
31) Head K, Gong S, Joseph S, Wang C, Burkhardt T, Rossi M, et al. Defining the expression pattern of the LGI1 gene in BAC trans-genic mice. Mamm Genome 2007; 18(5):328-37. doi: 10.1007/s00335-007-9024-6.
32) Bien C. Management of autoimmune encephalitis. Curr Opin Neurol 2021; 34(2):166-71. doi: 10.1097/WCO.0000000000000909.
33) Flanagan E, McKeon A, Lennon V, Boeve B, Trenerry M, Tan K, et al. Autoimmune dementia: clinical course and predictors of im-munotherapy response. Mayo Clin Proc 2010; 85(10):881-97. doi: 10.4065/mcp.2010.0326.
34) Lee W, Lee S, Byun J, Sunwoo J, Kim T, Lim J, et al. Rituximab treatment for autoimmune limbic encephalitis in an institutional co-hort. Neurology 2016; 86(18):1683-91. doi: 10.1212/WNL.0000000000002635.
35) Hermetter C, Fazekas F, Hochmeister S. Systematic review: syndromes, early diagnosis, and treatment in autoimmune enceph-alitis. Front Neurol 2018; 9:706. doi: 10.3389/fneur.2018.00706.
36) Deuschl C, Rüber T, Ernst L, Fendler W, Kirchner J, Mönning-hoff C, et al. 18F-FDG-PET/MRI in the diagnostic work-up of limbic encephalitis. PLoS One 2020; 15(1):e0227906. doi: 10.1371/journal.pone.0227906.
37) Gadoth A, Nisnboym M, Alcalay Y, Weinstein T. LGI1 enceph-alitis associated hyponatremia, is it all in the brain? Neurology 2021; 96(Suppl 15):e2256.
38) von Rhein B, Wagner J, Widman G, Malter M, Elger C, Helm-staedter C. Suspected antibody negative autoimmune limbic en-cephalitis: outcome of immunotherapy. Acta Neurol Scand 2017; 135(1):134-41. doi: 10.1111/ane.12575.
Publicado
2023-03-16
Cómo citar
1.
Orellano P, Perna Ramos A. Encefalitis límbica asociada a secreción inadecuada de hormona antidiurética. Rev. Méd. Urug. [Internet]. 16 de marzo de 2023 [citado 16 de abril de 2024];39(1):e701. Disponible en: http://www2.rmu.org.uy/ojsrmu311/index.php/rmu/article/view/1019
Sección
Casos Clínicos
