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Refractory Longitudinally Extensive Transverse Myelitis Responsive to Cyclophosphamide

  • Laura J. Baxter (a1), Shuo Chen (a1), Philippe Couillard (a1) (a2), James N. Scott (a1) (a3), Christopher J. Doig (a2) (a4), Fiona Costello (a1) (a4), Louis P. Girard (a5), John Klassen (a5) (a6) and Jodie M. Burton (a1) (a7)...

Abstract

Severe longitudinally extensive transverse myelitis (LETM) can cause quadriplegia, marked sensory dysfunction, and respiratory failure. Some patients are unresponsive to conventional immune therapy. We report two cases of severe immune-mediated LETM requiring intensive care admission that failed to respond to high-dose corticosteroids, plasma exchange, intravenous immunoglobulin, and rituximab. Disease cessation and significant recovery was achieved after cyclophosphamide induction. In patients with severe acute immune-mediated LETM who fail to respond to corticosteroids and plasma exchange, cyclophosphamide induction should be considered. This agent and regimen provides a robust immunosuppressive response and can be induced rapidly. Cyclophosphamide effects and supportive evidence are discussed.

Myélite transverse longitudinalement étendue répondant à la cyclophosphamide. La myélite transverse longitudinalement étendue (MTLE) peut causer une quadriplégie, une dysfonction sensitive importante et une insuffisance respiratoire. Certains patients ne répondent pas à l’immunothérapie conventionnelle. Nous rapportons deux cas de MTLE sévère d’origine auto-immune nécessitant une hospitalisation à l’unité des soins intensifs et ne réagissant pas au traitement par les corticostéroïdes à haute dose, à la plasmaphérèse, à l’immunoglobuline intraveineuse ou au rituximab. Le traitement par la cyclophosphamide a entraîné l’arrêt de la maladie et une récupération importante. Chez les patients qui présentent une MTLE auto-immune aiguë sévère qui ne répond pas aux corticostéroïdes et à la plasmaphérèse, le traitement par la cyclophosphamide devrait être envisagé. Cet agent provoque une réponse immunosuppressive importante qui peut être induite rapidement. Nous discutons des effets de la cyclophosphamide et des données appuyant son utilisation.

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Copyright

Corresponding author

Correspondence to: Laura J. Baxter, Adult Neurology Residency Program Office, Department of Clinical Neurosciences, 12th Floor, Foothills Medical Centre, 1403 – 29 Street NW, University of Calgary, Calgary, AB T2N 2T9. Email: laura.baxter@ahs.ca

References

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1. Greenberg, BM, Frohman, EM. Immune-mediated myelopathies. Continuum. 2015;21:121-131.
2. Scott, TF, Frohman, EM, De Seze, J, Gronseth, GS, Weinshenker, BG. Evidence-based guideline: clinical evaluation and treatment of transverse myelitis. Neurology. 2011;77:2128-2134.
3. Greenburg, GM, Thomas, KP, Krishnan, C, Kaplin, AI, Calabresi, PA, Kerr, DA. Idiopathic transverse myelitis: Corticosteroids, plasma exchange, or cyclophosphamide. Neurol. 2007;68:1614-1617.
4. Cree, BA, Lamb, S, Morgan, K, Chen, A, Waubant, E, Genain, C. An open label study of the effects of rituximab in neuromyelitis optica. Neurology. 2005;64:1270-1272.
5. Weiner, HL, Mackin, GA, Orav, EJ, et al. Intermittent cyclophosphamide pulse therapy in progressive multiple sclerosis: final report of the Northeast Cooperative Multiple Sclerosis Treatment Group. Neurology. 1993;43:910-918.
6. Partners, MS. Center. Cytoxan/methylprednisolone in-patient induction regimen physician’s orders. Available from: http://partnersmscenter.org/index.php?id=45&mn=5&sm=5-4.
7. Kim, S-H, Huh, S-Y, Lee, SJ, Joung, A, Kim, HJ. A 5-year follow-up of rituximab treatment in patients with neuromyelitis optica spectrum disorder. JAMA Neurol. 2013;70:1110-1117.
8. Stasi, R, Pagano, A, Stipa, E, Amadori, S. Rituximab chimeric anti-CD20 monoclonal antibody treatment for adults with chronic idiopathic thrombocytopenic purpura. Blood. 2001;98:952-957.
9. Ward, DM. Conventional apheresis therapies: a review. J Clin Apher. 2011;26:230-238.
10. Sackett, DL. Rules of evidence and clinical recommendations on the use of antithrombotic agents. Chest. 1989;95:2S-4S.
11. International Standards for Neurological Classification of SCI (ISNCSCI) Exam. Available from: http://asia-spinalinjury.org/wp-content/uploads/2016/02/International_Stds_Diagram_Worksheet.pdf.
12. Yaguchi, H, Sakushima, K, Takahashi, I, et al. Efficacy of intravenous cyclophosphamide therapy for neuromyelitis optica spectrum disorder. Intern Med. 2013;52:969-972.
13. Bichuetti, DB, Oliveira, EM, de Castro Boulos Fde, C, Gabbai, AA. Lack of response to pulse cyclophosphamide in neuromyelitis optica: evaluation of 7 patients. Arch Neurol. 2012;69:938-939.

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