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DOI: 10.1055/s-0040-1716824
Fulminant Acute Disseminated Encephalomyelitis: A Remarkable Outcome with Cyclophosphamide
Authors

Abstract
Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system which occurs predominantly in the pediatric population. Acute treatment is high-dose intravenous glucocorticoids. Alternative treatment is usually intravenous immune globulin and/or plasma exchange. Fulminant ADEM is rare in children. Only a few cases of cyclophosphamide use in refractory ADEM have been reported. Here, we report a case of a 12-year-old girl with fulminant ADEM who was comatose and improved dramatically after cyclophosphamide administration. Cyclophosphamide treatment should be considered as a therapy in children with fulminant ADEM nonresponsive to standard therapies.
Publikationsverlauf
Eingereicht: 12. Juni 2020
Angenommen: 09. August 2020
Artikel online veröffentlicht:
21. September 2020
© 2020. Thieme. All rights reserved.
Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany
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References
- 1 Erol I, Ozkale Y, Alkan O, Alehan F. Acute disseminated encephalomyelitis in children and adolescents: a single center experience. Pediatr Neurol 2013; 49 (04) 266-273
- 2 Leake JA, Albani S, Kao AS. et al. Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr Infect Dis J 2004; 23 (08) 756-764
- 3 Pavone P, Pettoello-Mantovano M, Le Pira A. et al. Acute disseminated encephalomyelitis: a long-term prospective study and meta-analysis. Neuropediatrics 2010; 41 (06) 246-255
- 4 Noorbakhsh F, Johnson RT, Emery D, Power C. Acute disseminated encephalomyelitis: clinical and pathogenesis features. Neurol Clin 2008; 26 (03) 759-780 , ix
- 5 Murthy SN, Faden HS, Cohen ME, Bakshi R. Acute disseminated encephalomyelitis in children. Pediatrics 2002; 110 (2 Pt 1): e21
- 6 Tenembaum S, Chitnis T, Ness J, Hahn JS. International Pediatric MS Study Group. Acute disseminated encephalomyelitis. Neurology 2007; 68 (16, Suppl 2): S23-S36
- 7 Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ. Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology 2001; 56 (10) 1308-1312
- 8 de Mol CL, Wong YYM, van Pelt ED. et al. Incidence and outcome of acquired demyelinating syndromes in Dutch children: update of a nationwide and prospective study. J Neurol 2018; 265 (06) 1310-1319
- 9 Yamaguchi Y, Torisu H, Kira R. et al. A nationwide survey of pediatric acquired demyelinating syndromes in Japan. Neurology 2016; 87 (19) 2006-2015
- 10 Xiong CH, Yan Y, Liao Z. et al. Epidemiological characteristics of acute disseminated encephalomyelitis in Nanchang, China: a retrospective study. BMC Public Health 2014; 14: 111
- 11
Chen Y,
Ma F,
Xu Y,
Chu X,
Zhang J.
Incidence of acute disseminated encephalomyelitis in the Jiangsu province of China,
2008-2011. Mult Scler J Exp Transl Clin 2015; 1: 2055217315594831
Reference Ris Wihthout Link
- 12 Krupp LB, Banwell B, Tenembaum S. International Pediatric MS Study Group. Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology 2007; 68 (16, Suppl 2): S7-S12
- 13 Krupp LB, Tardieu M, Amato MP. International Pediatric Multiple Sclerosis Study Group. et al; International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions. Mult Scler 2013; 19 (10) 1261-1267
- 14 Iype M, Kunju PAM, Saradakutty G, Anish TS, Sreedharan M, Ahamed SM. Short term outcome of ADEM: results from a retrospective cohort study from South India. Mult Scler Relat Disord 2017; 18: 128-134
- 15 Nishiyama M, Nagase H, Tomioka K. et al. Clinical time course of pediatric acute disseminated encephalomyelitis. Brain Dev 2019; 41 (06) 531-537
- 16 Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology 2002; 59 (08) 1224-1231
- 17 Wucherpfennig KW, Strominger JL. Molecular mimicry in T cell-mediated autoimmunity: viral peptides activate human T cell clones specific for myelin basic protein. Cell 1995; 80 (05) 695-705
- 18 Miller SD, McRae BL, Vanderlugt CL. et al. Evolution of the T-cell repertoire during the course of experimental immune-mediated demyelinating diseases. Immunol Rev 1995; 144: 225-244
- 19 Pohl-Koppe A, Burchett SK, Thiele EA, Hafler DA. Myelin basic protein reactive Th2 T cells are found in acute disseminated encephalomyelitis. J Neuroimmunol 1998; 91 (1-2): 19-27
- 20 Van Haren K, Tomooka BH, Kidd BA. et al. Serum autoantibodies to myelin peptides distinguish acute disseminated encephalomyelitis from relapsing-remitting multiple sclerosis. Mult Scler 2013; 19 (13) 1726-1733
- 21 Cole J, Evans E, Mwangi M, Mar S. Acute disseminated encephalomyelitis in children: an updated review based on current diagnostic criteria. Pediatr Neurol 2019; 100: 26-34
- 22 Straub J, Chofflon M, Delavelle J. Early high-dose intravenous methylprednisolone in acute disseminated encephalomyelitis: a successful recovery. Neurology 1997; 49 (04) 1145-1147
- 23 Sahlas DJ, Miller SP, Guerin M, Veilleux M, Francis G. Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin. Neurology 2000; 54 (06) 1370-1372
- 24 Khurana DS, Melvin JJ, Kothare SV. et al. Acute disseminated encephalomyelitis in children: discordant neurologic and neuroimaging abnormalities and response to plasmapheresis. Pediatrics 2005; 116 (02) 431-436
- 25 de Seze J, Debouverie M, Zephir H. et al. Acute fulminant demyelinating disease: a descriptive study of 60 patients. Arch Neurol 2007; 64 (10) 1426-1432
- 26 Yae Y, Kawano G, Yokochi T. et al. Fulminant acute disseminated encephalomyelitis in children. Brain Dev 2019; 41 (04) 373-377
- 27 Kim YH, Choi BK, Oh HS, Kang WJ, Mittler RS, Kwon BS. Mechanisms involved in synergistic anticancer effects of anti-4-1BB and cyclophosphamide therapy. Mol Cancer Ther 2009; 8 (02) 469-478
- 28 Smith DR, Balashov KE, Hafler DA, Khoury SJ, Weiner HL. Immune deviation following pulse cyclophosphamide/methylprednisolone treatment of multiple sclerosis: increased interleukin-4 production and associated eosinophilia. Ann Neurol 1997; 42 (03) 313-318
- 29 Brenton JN, Banwell BL. Therapeutic approach to the management of pediatric demyelinating disease: multiple sclerosis and acute disseminated encephalomyelitis. Neurotherapeutics 2016; 13 (01) 84-95
- 30 Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B. Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients. Neurology 2001; 56 (10) 1313-1318
- 31 Rothenberg J, Lastra A, Alvarez G, Irwin R. Acute disseminated encephalomyelitis responsive to cyclophosphamide therapy: a case report. J Med Cases 2015; 6: 290-294
- 32 Jaskowiak JM. Treatment of hemorrhagic acute disseminated encephalomyelitis with cyclophosphamide. Am J Health Syst Pharm 2016; 73 (04) 202-205
- 33 Ayed H, Chaudhary MW, AlBaradie R, Mir A. Use of cyclophosphamide in a child with fulminant acute disseminated encephalomyelitis. Child Neurol Open 2018; 5: 2329048x18754631
