A successful case of anti-NMDAR encephalitis without tumor treated with a prolonged regimen of plasmapheresis

Simabukuro, Mateus Mistieri; Watanabe, Rafael Gustavo Sato; Pinto, Lécio Figueira; Guariglia, Carla; Gonçalves, Daniella Costa de Menezes e; Anghinah, Renato

doi:10.1590/S1980-57642014DN81000014

ABSTRACT

Anti-NMDA receptor encephalitis is a severe but treatable autoimmune disease of the CNS. However, the use of immunotherapy and long-term outcomes have yet to be defined for this disease. We describe a case of an 18-year-old male diagnosed with anti-NMDAR encephalitis not associated with tumor, which did not respond to initial treatment with immunoglobulin, followed by corticosteroids, cyclophosphamide and evolved with significant clinical improvement after a prolonged course of plasmapheresis. Although it is not possible to affirm the good outcome was due solely to the prolonged plasmapheresis regimen, recently published data shows that improvement may take weeks or months to occur. This case discloses another therapeutic possibility for patients with refractory disease who fail to respond to recommended first-line and second-line therapy.

Key words: autoimmune disease; encephalitis; anti-NMDA receptor; plasmapheresis; psychiatric symptoms

RESUMO

A encefalite antirreceptor de NMDA é uma grave doença autoimune do sistema nervoso central, mas responsiva a tratamento. No entanto o uso da imunoterapia e o prognóstico a longo prazo ainda não foram estabelecidos para esta doença. Aqui nós descrevemos o caso de um homem de 18 anos de idade diagnosticado com encefalite antirreceptor de NMDA, não associada a tumor, o qual provavelmente não respondeu à terapia inicial com corticosteroides, ciclofosfamida e, o qual evoluiu com melhora clínica importante após um curso prolongado de plasmaférese. Embora não seja possível atribuir o desfecho favorável apenas ao regime de plasmaférese prolongado, uma vez que publicações recentes demonstram que a resposta à imunoterapia pode levar semanas à meses, nosso caso abre uma possibilidade terapêutica nova para casos com doença refratária, não responsivos ao tratamento de primeira e segunda-linha preconizados

Palavras-chave: doença autoimune; encefalite; receptor anti-NMDA; plasmaferese; sintomas psiquiátricos

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REFERENCES

¹ Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J. Paraneoplastic encephalitis, psychiatric symptoms, andhypoventilation in ovarian teratoma. Ann Neurol 2005;58:594-604.
² 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.
³ 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.
⁴ 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.
⁵ 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.
⁶ Pham HP, Daniel-Johnson JA, Stotler BA, Stephens H, Schwartz J. Therapeutic plasma exchange for the treatment of anti-NMDA receptor encephalitis. J Clin Apher. 2011;26:320-325.
⁷ Ishiura H, Matsuda S, Higashihara M, et al. Response of anti-NMDA receptor encephalitis without tumor to immunotherapy including rituximab. Neurology 2008;71:1921-1923.
⁸ Schimmel M, Bien CG, Vincent A, Schenk W, Penzien J. Successful treatment of anti-N-methyl-D-aspartate receptor encephalitis presenting with catatonia. Arch Dis Child 2009;94:314-316.
⁹ Agrawal S, Vincent A, Jacobson L, Milford D, Gupta R, Wassmer E. Successful treatment of antiN-methyl-d-aspartate receptor limbic encephalitis in a 22-monthold child with plasmapheresis and pharmacological immunomodulation. Arch Dis Child 2009;95:312.
¹⁰ Mirza MK, Pogoriler J, Paral K, et al. Adjunct therapeutic plasma exchange for anti-N-methyl-D-aspartate receptor antibody encephalitis:a case report and review of literature. J Clin Apher 2011;26:362-365.
¹¹ Szczepiorkowski ZM, Winters JL, Bandarenko N, et al. Guidelines on the use of therapeutic apheresis in clinical practice:evidence-based approach from the Apheresis Applications Committee of the American Society for Apheresis. J Clin Apher 2010;25:83-177.
¹² Pruss H, Dalmau J, Harms L, et al. Retrospective analysis of anti-glutamate receptor (type NMDA) antibodies in patients with encephalitis of unknown origin. Neurology 2010;75:1735-1739.
¹³ Davies G, Irani SR, Coltart C, et al. Anti-N-methyl-D-aspartate receptor antibodies:a potentially treatable cause of encephalitis in the intensive care unit. Crit Care Med 2010;38:679-682.

Publication Dates

Publication in this collection
Jan-Mar 2014

History

Received
21 Oct 2013
Accepted
20 Dec 2014

This is an open-access article distributed under the terms of the Creative Commons Attribution License

[1] ¹ Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J. Paraneoplastic encephalitis, psychiatric symptoms, andhypoventilation in ovarian teratoma. Ann Neurol 2005;58:594-604.

[2] ² 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.

[3] ³ 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.

[4] ⁴ 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.

[5] ⁵ 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.

[6] ⁶ Pham HP, Daniel-Johnson JA, Stotler BA, Stephens H, Schwartz J. Therapeutic plasma exchange for the treatment of anti-NMDA receptor encephalitis. J Clin Apher. 2011;26:320-325.

[7] ⁷ Ishiura H, Matsuda S, Higashihara M, et al. Response of anti-NMDA receptor encephalitis without tumor to immunotherapy including rituximab. Neurology 2008;71:1921-1923.

[8] ⁸ Schimmel M, Bien CG, Vincent A, Schenk W, Penzien J. Successful treatment of anti-N-methyl-D-aspartate receptor encephalitis presenting with catatonia. Arch Dis Child 2009;94:314-316.

[9] ⁹ Agrawal S, Vincent A, Jacobson L, Milford D, Gupta R, Wassmer E. Successful treatment of antiN-methyl-d-aspartate receptor limbic encephalitis in a 22-monthold child with plasmapheresis and pharmacological immunomodulation. Arch Dis Child 2009;95:312.

[10] ¹⁰ Mirza MK, Pogoriler J, Paral K, et al. Adjunct therapeutic plasma exchange for anti-N-methyl-D-aspartate receptor antibody encephalitis:a case report and review of literature. J Clin Apher 2011;26:362-365.

[11] ¹¹ Szczepiorkowski ZM, Winters JL, Bandarenko N, et al. Guidelines on the use of therapeutic apheresis in clinical practice:evidence-based approach from the Apheresis Applications Committee of the American Society for Apheresis. J Clin Apher 2010;25:83-177.

[12] ¹² Pruss H, Dalmau J, Harms L, et al. Retrospective analysis of anti-glutamate receptor (type NMDA) antibodies in patients with encephalitis of unknown origin. Neurology 2010;75:1735-1739.

[13] ¹³ Davies G, Irani SR, Coltart C, et al. Anti-N-methyl-D-aspartate receptor antibodies:a potentially treatable cause of encephalitis in the intensive care unit. Crit Care Med 2010;38:679-682.