Skip to main content

Advertisement

Log in

Differences in treatment of anti-NMDA receptor encephalitis: results of a worldwide survey

  • Original Communication
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

The objective of the study was to identify differences in treatment strategies for anti-NMDA receptor encephalitis based on specialty of treating physicians, geographic location, and years in practice. We conducted an anonymous worldwide electronic survey through the Practice Current section of Neurology® Clinical Practice to appraise differences in decisions about first- and second-line treatment and timing for initiation of second-line treatment for anti-NMDA receptor encephalitis. 399 participants answered all questions of the survey and were included in the analysis. 261 (65%) were adult neurologists, 86 (22%) were neurologists treating children, and 52 (13%) were pediatric rheumatologists. 179 (45%) responders practiced in the US. The majority agreed on the use of steroids and/or IVIg for first-line therapy and rituximab alone as second line. Differences in initial treatment regimen based on specialty included increased use of plasma exchange by adult neurologists (27%) and rituximab by pediatric rheumatologists (29%) (χ 2(4) = 27.43, p < 0.001). Trainees opted for plasma exchange (35%) and junior faculty picked rituximab (15%) more as part of first line (χ 2(4) = 13.37, p = 0.010). There was greater usage of anti-metabolites for second-line therapy outside of the US (15%) (χ 2(4) = 11.67, p = 0.020). US physicians also utilized second-line treatment earlier than their mostly European counterparts (14 vs. 23% used later than 2 weeks; χ 2(1) = 4.96, p = 0.026). Although treatment patterns were similar, differences observed across specialties and geographic locations may guide the development of consensus-driven guidelines by multi-disciplinary task forces. These guidelines may promote treatment trials of immunomodulators in autoimmune encephalitides.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Granerod J, Ambrose HE, Davies NWS, Clewley JP, Walsh AL, Morgan D et al (2010) Causes of encephalitis and differences in their clinical presentations in England: a multicentre, population-based prospective study. Lancet Infect Dis 10:835–844

    Article  PubMed  Google Scholar 

  2. Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J (2005) Paraneoplastic encephalitis, psychiatric symptoms, and hypoventilation in ovarian teratoma. Ann Neurol 58:594–604

    Article  PubMed  PubMed Central  Google Scholar 

  3. Dalmau J, Tuzun E, Wu HY, Masjuan J, Rossi JE, Voloschin A et al (2007) Paraneoplastic anti-N-methyl-d-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 61:25–36

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M et al (2008) Anti-NMDA receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 7:1091–1098

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T 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  CAS  PubMed  PubMed Central  Google Scholar 

  6. Gronseth GS, Woodroffe LM, Getchius TS (2011) Clinical practice guideline process manual. American Academy of Neurology, St. Paul

    Google Scholar 

  7. Bartolini L (2016) Practice current: how do you treat anti-NMDA receptor encephalitis? Neurol Clin Pract 6:69–72

    Article  Google Scholar 

  8. Irani SR, Bera K, Waters P, Zuliani L, Maxwell S, Zandi MS et al (2010) N-methyl-d-aspartate antibody encephalitis: temporal progression of clinical and paraclinical observations in a predominantly non-paraneoplastic disorder of both sexes. Brain 133:1655–1667

    Article  PubMed  PubMed Central  Google Scholar 

  9. Practice current. http://cp.neurology.org/site/misc/practice_current.xhtml. Accessed 12 Dec 2016

  10. Talan J (2016) When guidelines aren’t available, how do you treat…?: neurology turns to clinical practice surveys. Neurol Today 16(4):33–34

    Article  Google Scholar 

  11. Gastaldi M, Thouin A, Vincent A (2016) Antibody-mediated autoimmune encephalopathies and immunotherapies. Neurotherapeutics 13:147–162

    Article  CAS  PubMed  Google Scholar 

  12. Martinez-Hernandez E, Horvath J, Shiloh-Malawsky Y, Sangha N, Martinez-Lage M, Dalmau J (2011) Analysis of complement and plasma cells in the brain of patients with anti-NMDAR encephalitis. Neurology 77:589–593

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Rath E, Zwerina J, Oppl B, Nell-Duxneuner V (2015) Efficacy and safety of rituximab in rheumatic diseases. Wien Med Wochenschr 165:28–35

    Article  PubMed  Google Scholar 

  14. Tambralli A, Beukelman T, Cron RQ, Stoll ML (2015) Safety and efficacy of rituximab in childhood-onset systemic lupus erythematosus and other rheumatic diseases. J Rheumatol 42:541–546

    Article  CAS  PubMed  Google Scholar 

  15. Kosmidis ML, Dalakas MC (2010) Practical considerations on the use of rituximab in autoimmune neurological disorders. Ther Adv Neurol Disord 3:93–105

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Brummaier T, Pohanka E, Studnicka-Benke A, Pieringer H (2013) Using cyclophosphamide in inflammatory rheumatic diseases. Eur J Intern Med 24:590–596

    Article  CAS  PubMed  Google Scholar 

  17. Meuwissen SG, Ewe K, Gassull MA, Geboes K, Jewell D, Pallone F et al (2000) IOIBD questionnaire on the clinical use of azathioprine, 6-mercaptopurine, cyclosporin A and methotrexate in the treatment of inflammatory bowel diseases. Eur J Gastroenterol Hepatol 12:13–18

    Article  CAS  PubMed  Google Scholar 

  18. Kurian M, Fluss J, Korff C (2012) Anti-NMDA receptor encephalitis: the importance of early diagnosis and aggressive immunotherapy in tumor negative pediatric patients. Eur J Paediatr Neurol 16:764–765

    Article  PubMed  Google Scholar 

  19. Friedman CP, Gatti GG, Franz TM, Murphy GC, Wolf FM, Heckerling PS et al (2005) Do physicians know when their diagnoses are correct? Implications for decision support and error reduction. J Gen Intern Med 20:334–339

    Article  PubMed  PubMed Central  Google Scholar 

  20. Moulton CA, Regehr G, Mylopoulos M, MacRae HM (2007) Slowing down when you should: a new model of expert judgment. Acad Med 82:S109–S116

    Article  PubMed  Google Scholar 

  21. Fricker RD (2012) Sampling Methods for Web and E-mail Surveys. In: Fielding N, Lee RM, Blank G (eds) The SAGE handbook of online research methods. SAGE Publications, London

    Google Scholar 

Download references

Acknowledgements

The authors would like to thank the managing editor of Neurology Clinical Practice, Mrs. Sharon Quimby, for her contribution to the creation of the electronic survey.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Luca Bartolini.

Ethics declarations

Ethical standards

The present study was certified as research exempt from IRB review by Children’s National Health System Institutional Review Board Committee in accordance with article 45 CFR 46.101(b)—Category 2 (Research involving the use of educational tests, survey procedures, interview procedures or observation of public behavior). The manuscript does not contain clinical studies or patient data.

Conflicts of interest

The authors declare that they have no conflict of interest.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (DOCX 15 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Bartolini, L., Muscal, E. Differences in treatment of anti-NMDA receptor encephalitis: results of a worldwide survey. J Neurol 264, 647–653 (2017). https://doi.org/10.1007/s00415-017-8407-1

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-017-8407-1

Keywords

Navigation