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Therapeutisches Management von Autoimmun-Enzephalitiden

Therapeutic management of autoimmune encephalitides

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Zusammenfassung

Ein entscheidender Fortschritt der letzten Jahre war die Entdeckung von antineuralen Antikörpern mit Oberflächenantigenen bei Patienten mit Autoimmun-Enzephalitiden. Diese sind nicht nur syndromspezifisch, sondern sie verheißen auch ein Ansprechen auf Immuntherapien. Weiterhin gibt es aber auch Antikörper gegen intrazelluläre Targets oder Patienten ohne distinkte Antikörper. Diese sind wegen ihrer geringeren Spezifität oder Therapieresponsivität als „graue Fälle“ zu bewerten. Bei den hochwertigen Antikörpern sollte nach einer First-Line-Therapie mit Steroiden, intravenösen Immunglobulinen oder Apherese (allein oder in Kombination) eine Second-Line-Therapie mit Cyclophosphamid und/oder Rituximab erfolgen. Die „grauen Fälle“ sollten einen dreimonatigen Therapieversuch mit oralem Prednisolon erhalten.

Abstract

A decisive advance in recent years was the discovery of antineural antibodies with surface antigens in patients with autoimmune encephalitis. They are not only syndrome specific, but also predict a response to immunotherapy. On the other hand, there are antibodies against intracellular targets or patients without any distinct antibodies. Due to their lower specificity or poorer responsiveness to immunotherapies, these are considered “gray cases”. In “high-rank” antibodies, first-line therapy consists of steroids, intravenous immunoglobulins or apheresis (alone or in combination). In the case of insufficient response, a second-line therapy with cyclophosphamide and/or rituximab is recommended. “Gray cases” should receive at least a three-month course of oral prednisolone.

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Literatur

  1. Amato AA, Griggs RC (2003) Treatment of idiopathic inflammatory myopathies. Curr Opin Neurol 16:569–575

    Article  CAS  PubMed  Google Scholar 

  2. Beck RW, Cleary PA, Anderson MM Jr. et al (1992) A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. The Optic Neuritis Study Group. N Engl J Med 326:581–588

    Article  CAS  PubMed  Google Scholar 

  3. Bien CG, Urbach H, Schramm J et al (2007) Limbic encephalitis as a precipitating event in adult-onset temporal lobe epilepsy. Neurology 69:1236–1244

    Article  CAS  PubMed  Google Scholar 

  4. Bien CG, Vincent A, Barnett MH et al (2012) Immunopathology of autoantibody-associated encephalitides: clues for pathogenesis. Brain 135:1622–1638

    Article  PubMed  Google Scholar 

  5. Carvajal-Gonzalez A, Leite MI, Waters P et al (2014) Glycine receptor antibodies in PERM and related syndromes: characteristics, clinical features and outcomes. Brain 137:2178–2192

    Article  PubMed Central  PubMed  Google Scholar 

  6. Castillo P, Woodruff B, Caselli R et al (2006) Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Arch Neurol 63:197–202

    Article  PubMed  Google Scholar 

  7. Dalakas MC (1991) Polymyositis, dermatomyositis and inclusion-body myositis. N Engl J Med 325:1487–1498

    Article  CAS  PubMed  Google Scholar 

  8. Dalmau J, Lancaster E, Martinez-Hernandez E et al (2011) Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 10:63–74

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  9. DeSena AD, Noland DK, Matevosyan K et al (2015) Intravenous methylprednisolone versus therapeutic plasma exchange for treatment of anti-n-methyl-d-aspartate receptor antibody encephalitis: A retrospective review. J Clin Apher (in press)

  10. Dogan Onugoren M, Deuretzbacher D, Haensch CA et al (2014) Limbic encephalitis due to GABAB and AMPA receptor antibodies: a case series. J Neurol Neurosurg Psychiatry (in press)

  11. Gabilondo I, Saiz A, Galan L et al (2011) Analysis of relapses in anti-NMDAR encephalitis. Neurology 77:996–999

    Article  CAS  PubMed  Google Scholar 

  12. Gold R, Leitliniengruppe (2014) Diagnose und Therapie der Multiplen Sklerose. In: Diener HC, Weimar C (Hrsg) Leitlinien für Diagnostik und Therapie in der Neurologie (http://www.dgn.org/leitlinien/2333-ll-31-2012-diagnose-und-therapie-der-multiplen-sklerose. Zugegriffen: 31.03.2015

    Google Scholar 

  13. Graus F, Saiz A, Dalmau J (2010) Antibodies and neuronal autoimmune disorders of the CNS. J Neurol 257:509–517

    Article  CAS  PubMed  Google Scholar 

  14. Höftberger R, Titulaer MJ, Sabater L et al (2013) Encephalitis and GABAB receptor antibodies: novel findings in a new case series of 20 patients. Neurology 81:1500–1506

    Article  PubMed Central  PubMed  Google Scholar 

  15. Irani SR, Gelfand JM, Bettcher BM et al (2014) Effect of rituximab in patients with leucine-rich, glioma-inactivated 1 antibody-associated encephalopathy. JAMA Neurol 71:896–900

    Article  PubMed Central  PubMed  Google Scholar 

  16. Irani SR, Stagg CJ, Schott JM et al (2013) Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype. Brain 136:3151–3162

    Article  PubMed  Google Scholar 

  17. Jaben EA, Winters JL (2012) Plasma exchange as a therapeutic option in patients with neurologic symptoms due to antibodies to voltage-gated potassium channels: a report of five cases and review of the literature. J Clin Apher 27:267–273

    Article  PubMed  Google Scholar 

  18. Keime-Guibert F, Graus F, Broet P et al (1999) Clinical outcome of patients with anti-Hu-associated encephalomyelitis after treatment of the tumor. Neurology 53:1719–1723

    Article  CAS  PubMed  Google Scholar 

  19. Keime-Guibert F, Graus F, Fleury A et al (2000) Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (Anti-Hu, anti-Yo) with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone. J Neurol.Neurosurg. Psychiatry 68:479–482

    CAS  Google Scholar 

  20. Kerling F, Bl Mcke I, Stefan H (2008) Pitfalls in diagnosing limbic encephalitis – a case report. Acta Neurol Scand 118:339–342

    Article  CAS  PubMed  Google Scholar 

  21. Köhler W, Ehrlich S, Dohmen C et al (2015) Tryptophan immunoadsorption for the treatment of autoimmune encephalitis. Eur J Neurol 22:203–206

    Article  PubMed  Google Scholar 

  22. Kruse JL, Jeffrey JK, Davis MC et al (2014) Anti-N-methyl-D-aspartate receptor encephalitis: a targeted review of clinical presentation, diagnosis, and approaches to psychopharmacologic management. Ann Clin Psychiatry 26:111–119

    PubMed  Google Scholar 

  23. Laxer RM, Stein LD, Petty RE (1987) Intravenous pulse methylprednisolone treatment of juvenile dermatomyositis. Arthritis Rheum 30:328–334

    Article  CAS  PubMed  Google Scholar 

  24. Malter MP, Helmstaedter C, Urbach H et al (2010) Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis. Annals of Neurology 67:470–478

    Article  PubMed  Google Scholar 

  25. Meinck HM (2013) Das Stiff-Man-Syndrom und seine Varianten. Nervenarzt 84:450–454

    Article  PubMed  Google Scholar 

  26. Muehlebner A, Groeppel G, Pahs G et al (2010) Beneficial effect of epilepsy surgery in a case of childhood non-paraneoplastic limbic encephalitis. Epilepsy Res 90:295–299

    Article  PubMed  Google Scholar 

  27. Schimmel M, Bien CG, Vincent A et al (2009) Successful treatment of anti-N-methyl-D-aspartate receptor encephalitis presenting with catatonia. Arch Dis Child 94:314–316

    Article  CAS  PubMed  Google Scholar 

  28. Titulaer MJ, Mccracken L, Gabilondo I et al (2013) Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 12:157–165

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  29. Vincent A, Bien CG, Irani SR et al (2011) Autoantibodies associated with diseases of the CNS: new developments and future challenges. Lancet Neurol 10:759–772

    Article  CAS  PubMed  Google Scholar 

  30. Wetzels JF (2004) Cyclophosphamide-induced gonadal toxicity: a treatment dilemma in patients with lupus nephritis? Neth J Med 62:347–352

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Epilepsie-Zentrum Bethel, Krankenhaus Mara, Maraweg 17–21, 33617, Bielefeld, Deutschland

    C. G. Bien & C. Bien

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  1. C. G. Bien
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Correspondence to C. G. Bien.

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Interessenkonflikt

Der Arbeitgeber von C. G. Bien und C. Bien (Krankenhaus Mara, Bielefeld) betreibt ein Labor für den Nachweis von Autoantikörpern einschließlich der in diesem Beitrag erwähnten Antikörper; externe Einsender bezahlen Gebühren für die Antikörperdiagnostik. C. G. Bien wirkte in Advisory Boards von Eisai (Frankfurt) und UCB (Monheim) mit, erhielt Reiseunterstützung von Eisai (Frankfurt), UCB (Monheim), Desitin (Hamburg) und Grifols (Frankfurt), erhielt Honorare für Fortbildungsvorträge von Eisai (Frankfurt), UCB (Monheim), Desitin (Hamburg), Diamed (Köln) und Fresenius Medical Care (Bad Homburg) und erhielt Forschungsunterstützung von Astellas Pharma (München), Octapharma (Langenfeld), Diamed (Köln) und Fresenius Medical Care (Bad Homburg).

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Bien, C.G., Bien, C. Therapeutisches Management von Autoimmun-Enzephalitiden. Z. Epileptol. 28, 201–206 (2015). https://doi.org/10.1007/s10309-015-0006-5

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  • DOI: https://doi.org/10.1007/s10309-015-0006-5

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