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Subacute Sclerosing Panencephalitis

  • Marjo S. van der Knaap
  • Jacob Valk

Abstract

Subacute sclerosing panencephalitis (SSPE) is one of the slow virus infections of the CNS. It is a rare disorder, but it is the commonest of the chronic virus infections to affect children. It is caused by measles virus. SSPE is a disease of childhood and adolescence, with an age range of 4–20 years and a peak incidence at 7–10 years. There is a racial difference in incidence of SSPE: in the United States the incidence is four times higher in Whites than in Blacks. Boys outnumber girls by 3 or 2 to 1. Children who acquire measles infection under 2 years of age are more likely to develop SSPE. The mean age of infection is 12–14 months. In exceptional cases SSPE can also occur after live measles immunization, but the rate of incidence is highly reduced after immunization. Since the introduction of measles vaccination, and since coverage in The Netherlands has reached 95%, the incidence of SSPE has gone down to one case annually. In Japan, 10–25 new cases a year are reported.

Keywords

White Matter Lesion Measle Virus Measle Vaccination Acute Disseminate Encephalomyelitis Subacute Sclerosing Panencephalitis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. Beersma MF, Galama JMD, van Druten HAM, Renier WO, Lucas CJ, Kapsenberg JG (1992) Subacute sclerosing panencephalitis in the Netherlands–1976–1990 Int J Epidemiol 21: 583–588Google Scholar
  2. Bohlega S, Al-Kawi MZ (1994) Subacute sclerosing panencephalitis. J Neuroimag 4: 71–76Google Scholar
  3. Brown HR, Goller NL, Rudelli RD, Dymecki J, Wisniewski HM (1989) Postmortem detection of measles virus in non-neural tissues in subacute sclerosing panencephalitis. Ann Neurol 26: 263–268PubMedCrossRefGoogle Scholar
  4. Choppin PW (1981) Measles virus and chronic neurological diseases. Ann Neurol 9: 17–20PubMedCrossRefGoogle Scholar
  5. Dhib-Jalbut S, Haddad FS (1984) Subacute sclerosing panencephalitis in one member of identical twins. Neuropediatrics 15: 49–51PubMedCrossRefGoogle Scholar
  6. Dyken PR (1985) Subacute sclerosing panencephalitis. Neurol Clin 3: 179–196PubMedGoogle Scholar
  7. Dyken PR, Cunningham SC, Charles Ward L (1989) Changing character of subacute sclerosing panencephalitis in the United States. Pediatr Neurol 5: 339–341PubMedCrossRefGoogle Scholar
  8. Fournier JG, Tardieu M, Lebon P, Robain O, Ponsot G, Rozenblatt S, Bouteille M (1985) Detection of measles virus RNA in lymphocytes from peripheral blood and brain perivascular infiltrates of patients with subacut sclerosing panencephalitis. N Engl J Med 313: 910–915PubMedCrossRefGoogle Scholar
  9. Geller TJ, Vern BA, Sarwar M (1987) Focal MRI findings in early SSPE. Pediatr Neurol 3: 310–312PubMedCrossRefGoogle Scholar
  10. Griffith JF (1985) Subacute sclerosing panencephalitis and lymphocytes. N Engl J Med 313: 952–954PubMedCrossRefGoogle Scholar
  11. Hall WW, Choppin PW (1981) Measles-virus proteins in the brain tissue of patients with subacute sclerosing panencephalitis. N Engl J Med 304: 1152–1155PubMedCrossRefGoogle Scholar
  12. Iwasaki Y, Sako K, Tsunoda I, Ohara Y (1993) Phenotypes of mononuclear cell infiltrates in human central nervous system. Acta Neuropathol (Berl) 85: 653–657CrossRefGoogle Scholar
  13. Jabbour JT, Garcia JH, Lemmi H, Ragland J, Duenas DA, Sever JL (1969) Subacute sclerosing panencephalitis. JAMA 207: 2248–2254PubMedCrossRefGoogle Scholar
  14. Johnson KP, Byington DP, Gaddis L (1974) Subacute sclerosing panencephalitis. Adv Neurol 6: 77–86PubMedGoogle Scholar
  15. Lum GB, Williams JP, Dyken PR, Machen BC, Dotson PM, Harpen MD, McLeod N (1986) Magnetic resonance and CT imaging correlated with clinical status in SSPE. Pediatr Neurol 2: 75–79PubMedCrossRefGoogle Scholar
  16. Modi G, Campbell H, Bill P (1989) Subacute sclerosing panencephalitis: changes on CT scan during acute relapse. Neuro-radiology 31: 433–434Google Scholar
  17. Noetzel MJ, Dodson WE (1983) Progressive CT abnormalities despite clinical improvement in SSPE treated with inosiplex. Ann Neurol 13: 457–460PubMedCrossRefGoogle Scholar
  18. Ohya T, Martinez AJ, Jabbour JT, Lemmi, Duenas DA (1974) Subacute sclerosing panencephalitis. Neurology 24: 211–218CrossRefGoogle Scholar
  19. Payne FE, Baublis JV, Itabashi HH (1969) Isolation of measles virus from cell cultures of brain from a patient with subacute sclerosing panencephalitis. N Engl J Med 281: 585–589PubMedCrossRefGoogle Scholar
  20. Poser CM (1990) Notes on the pathogenesis of subacute sclerosing panencephalitis. J Neurol Sci 95: 219–224PubMedCrossRefGoogle Scholar
  21. Riekkinen P, Palo J, Arstila A, Rinne UK, Frey H, Savolainen H, Kivalo E (1970) Protein composition of white matter myelin in subacute sclerosing panencephalits. J Neurol Sci 14: 15–20CrossRefGoogle Scholar
  22. Scully RE, Mark EJ, McNeely BU (1986) Case records of the masschusetts general hospital. N Engl J Med 314: 1689–1700CrossRefGoogle Scholar
  23. Takayama S, Iwasaki Y, Yamanouchi H, Sugai K, Takashima S, Iwasaki A (1994) Characteristic clinical features in a case of fulminant subacute sclerosing panencephalitis. Brain Dev 16: 132–135PubMedCrossRefGoogle Scholar
  24. Tan E, Namer IJ, Ciger A, Zilch T, Kucukali T (1991) The prognosis of subacute sclerosing panencephalitis in adults. Clin Neurol Neurosurg 93–3: 205–209CrossRefGoogle Scholar
  25. Ter Meulen V, Hall WW (1978) Slow virus infections of the nervous system:virological immunological and pathogenetic considerations. J Gen Virol 41: 1–25PubMedCrossRefGoogle Scholar
  26. Tsuchiya K, Yamauchi T, Furui S, Suda Y, Takenaka E (1988) MR imaging vs CT in subacute sclerosing panencephalitis. AJNR 9: 943–946PubMedGoogle Scholar
  27. Winer JB, Pires M, Kermode A, Ginsberg L, Rossor M (1991) Resolving MRI abnormalities with progression of subacute sclerosing panencephalitis. Neuroradiology 33: 178–180PubMedCrossRefGoogle Scholar
  28. Yagi S, Miura Y, Mizuta S, Wakunami A, Kataoka N, Morita T, Morita K, Ono S, Fukunaga M (1993) Chronological SPECT studies of a patient with subacute sclerosing panencephalitis. Brain Dev 15: 141–145PubMedCrossRefGoogle Scholar
  29. Yalaz K, Anlar B, Oktem F, Aysun S, Ustacelebi S, Gurcay O, Gurcay O, Gucuyener K, Renda Y (1992) Intraventricular interferon and oral inosiplex in the treatment of subacute sclerosing panencephalitis. Neurology 42: 488–491PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1995

Authors and Affiliations

  • Marjo S. van der Knaap
    • 1
  • Jacob Valk
    • 2
  1. 1.Department of Child NeurologyFree University HospitalAmsterdamThe Netherlands
  2. 2.Department of Diagnostic RadiologyFree University HospitalAmsterdamThe Netherlands

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