Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A long-term subacute sclerosing panencephalitis survivor treated with intraventricular interferon-alpha for 13 years

  • Kwak, Minsun (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Yeh, Hye-Ryun (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Yum, Mi-Sun (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Kim, Hyun-Jin (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • You, Su Jeong (Department of Pediatrics, Inje University Sanggye Paik Hospital, Inje University College of Medicine) ;
  • Ko, Tae-Sung (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine)
  • Received : 2018.06.26
  • Accepted : 2018.09.04
  • Published : 2019.03.15
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Abstract

Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, and fatal central nervous system disorder resulting from persistent measles virus infection. Long-term data are scarce, with a maximum follow-up period of 10 years. Interferon-alpha ($IFN-{\alpha}$) is a protein that exerts its antiviral activity via enhancement of cellular immune response and is reported to be an effective drug for the treatment of SSPE. However, there is currently no consensus regarding the optimal duration of $IFN-{\alpha}$ therapy. Here, we present a case report of a patient with SSPE treated with long-term intraventricular $IFN-{\alpha}$ therapy, which facilitated clinical improvement and neurological stabilization without causing serious adverse effects. To the best of our knowledge, this is one of the longest follow-up studies investigating a patient with SSPE receiving intraventricular $IFN-{\alpha}$ treatment. Further studies are necessary to validate the benefits and safety of long-term intraventricular $IFN-{\alpha}$ treatment in patients with SSPE.

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References

  1. Prashanth LK, Taly AB, Ravi V, Sinha S, Rao S. Long term survival in subacute sclerosing panencephalitis: an enigma. Brain Dev 2006; 28:447-52. https://doi.org/10.1016/j.braindev.2006.01.008
  2. Barraclough CR. Long-term survival of a case of subacute sclerosing panencephalitis. J Neurol Neurosurg Psychiatry 1983;46:457-8. https://doi.org/10.1136/jnnp.46.5.457
  3. Risk WS, Haddad FS. The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the Middle East. Arch Neurol 1979;36:610-4. https://doi.org/10.1001/archneur.1979.00500460044004
  4. Anlar B, Yalaz K, Oktem F, Kose G. Long-term follow-up of patients with subacute sclerosing panencephalitis treated with intraventricular alpha-interferon. Neurology 1997;48:526-8. https://doi.org/10.1212/WNL.48.2.526
  5. Miyazaki M, Nishimura M, Toda Y, Saijo T, Mori K, Kuroda Y. Long-term follow-up of a patient with subacute sclerosing panencephalitis successfully treated with intrathecal interferon alpha. Brain Dev 2005; 27:301-3. https://doi.org/10.1016/j.braindev.2004.07.003
  6. Jabbour JT, Garcia JH, Lemmi H, Ragland J, Duenas DA, Sever JL. Subacute sclerosing panencephalitis. A multidisciplinary study of eight cases. JAMA 1969;207:2248-54. https://doi.org/10.1001/jama.1969.03150250078007
  7. Gascon GG; International Consortium on Subacute Sclerosing Panencephalitis. Randomized treatment study of inosiplex versus combined inosiplex and intraventricular interferon-alpha in subacute sclerosing panencephalitis (SSPE): international multicenter study. J Child Neurol 2003;18:819-27. https://doi.org/10.1177/088307380301801201
  8. Wendorf KA, Winter K, Zipprich J, Schechter R, Hacker JK, Preas C, et al. Subacute sclerosing panencephalitis: the devastating measles complication that might be more common than previously estimated. Clin Infect Dis 2017;65:226-32. https://doi.org/10.1093/cid/cix302
  9. Fukuyama Y, Nihei K, Matsumoto S, Ebina T, Kamoshita S, Sato T, et al. Clinical effects of MND-19 (Inosiplex) on subacute sclerosing panencephalitis--a multi-institutional collaborative study--The Inosiplex-SSPE Research Committee. Brain Dev 1987;9:270-82. https://doi.org/10.1016/S0387-7604(87)80044-X
  10. Gascon G, Yamani S, Crowell J, Stigsby B, Nester M, Kanaan I, et al. Combined oral isoprinosine-intraventricular alpha-interferon therapy for subacute sclerosing panencephalitis. Brain Dev 1993;15:346-55. https://doi.org/10.1016/0387-7604(93)90120-W
  11. Aydin OF, Senbil N, Kuyucu N, Gurer YK. Combined treatment with subcutaneous interferon-alpha, oral isoprinosine, and lamivudine for subacute sclerosing panencephalitis. J Child Neurol 2003;18:104-8. https://doi.org/10.1177/08830738030180020701
  12. Kuskonmaz B, Uckan D, Yalnizoglu D, Gunel M, Karli Oguz K, Konuskan B, et al. Mesenchymal stem cell application in children with subacute sclerosing panencephalitis. Dev Med Child Neurol 2015; 57:880-3. https://doi.org/10.1111/dmcn.12747
  13. Anlar B, Aydin OF, Guven A, Sonmez FM, Kose G, Herguner O. Retrospective evaluation of interferon-beta treatment in subacute sclerosing panencephalitis. Clin Ther 2004;26:1890-4. https://doi.org/10.1016/j.clinthera.2004.11.002
  14. Tomoda A, Shiraishi S, Hosoya M, Hamada A, Miike T. Combined treatment with interferon-alpha and ribavirin for subacute sclerosing panencephalitis. Pediatr Neurol 2001;24:54-9. https://doi.org/10.1016/S0887-8994(00)00233-2
  15. Hosoya M, Shigeta S, Mori S, Tomoda A, Shiraishi S, Miike T, et al. High-dose intravenous ribavirin therapy for subacute sclerosing panencephalitis. Antimicrob Agents Chemother 2001;45:943-5. https://doi.org/10.1128/AAC.45.3.943-945.2001

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