Skip to main content
Postgraduate Medical Journal logoLink to Postgraduate Medical Journal
. 1969 Jun;45(524):401–407. doi: 10.1136/pgmj.45.524.401

Subacute sclerosing panencephalitis: measles encephalitis of temperate evolution

A D Dayan
PMCID: PMC2466721  PMID: 5798690

Abstract

Subacute sclerosing panencephalitis is a rare disorder of late childhood and early adolescence Affected patients usually show behavioural and intellectual disturbance and involuntary movements before dying in coma after about 12 months. At some stage most have characteristic electroencephalographic abnormalities. Pathologically, changes in the brain are those of subacute encephalitis with a variable gliosis of the white matter, and sometimes intranuclear inclusion bodies in neurones and glial cells.

Recent studies in many patients have shown high levels of circulating anti-measles antibodies, measles antigen in cells in the brain, and sometimes, myxo-virus filaments in cells there. These findings suggest that SSPE may be a slow measles virus infection of the nervous system. Possible explanations for the slow evolution of the encephalitis include disordered immune mechanisms and intracellular persistence of virus in a defective phase.

Full text

PDF
401

Images in this article

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Adams J. M., Baird C., Filloy L. Inclusion bodies in measles encephalitis. JAMA. 1966 Jan 24;195(4):290–298. [PubMed] [Google Scholar]
  2. Adels B. R., Gajdusek C., Gibbs C. J., Jr, Albrecht P., Rogers N. G. Attempts to transmit subacute sclerosing panencephalitis and isolate a measles related agent, with a study of the immune response in patients and experimental animals. Neurology. 1968 Jan;18(1 Pt 2):30–51. doi: 10.1212/wnl.18.1_part_2.030. [DOI] [PubMed] [Google Scholar]
  3. CANAL N., TORCK P. AN EPIDEMIOLOGICAL STUDY OF SUB-ACUTE SCLEROSING LEUCOENCEPHALITIS IN BELGIUM. J Neurol Sci. 1964 Jul-Aug;1(4):380–389. doi: 10.1016/0022-510x(64)90015-2. [DOI] [PubMed] [Google Scholar]
  4. Cobb W. A., Morgan-Hughes J. A. Non-fatal subacute sclerosing leucoencephalitis. J Neurol Neurosurg Psychiatry. 1968 Apr;31(2):115–123. doi: 10.1136/jnnp.31.2.115. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Cobb W. The periodic events of subacute sclerosing leucoencephalitis. Electroencephalogr Clin Neurophysiol. 1966 Sep;21(3):278–294. doi: 10.1016/0013-4694(66)90077-0. [DOI] [PubMed] [Google Scholar]
  6. Connolly J. H., Allen I. V., Hurwitz L. J., Millar J. H. Measles-virus antibody and antigen in subacute sclerosing panencephalitis. Lancet. 1967 Mar 11;1(7489):542–544. doi: 10.1016/s0140-6736(67)92117-4. [DOI] [PubMed] [Google Scholar]
  7. Cutler R. W., Watters G. V., Hammerstad J. P., Merler E. Origin of cerebrospinal fluid gamma globulin in subacute sclerosing leukoencephalitis. Arch Neurol. 1967 Dec;17(6):620–628. doi: 10.1001/archneur.1967.00470300062011. [DOI] [PubMed] [Google Scholar]
  8. Dawson J. R. Cellular Inclusions in Cerebral Lesions of Lethargic Encephalitis. Am J Pathol. 1933 Jan;9(1):7–16.3. [PMC free article] [PubMed] [Google Scholar]
  9. Dayan A. D., Gostling J. V., Greaves J. L., Stevens D. W., Woodhouse M. A. Evidence of a pseudomyxovirus in the brain in subacute sclerosing leucoencephalitis. Lancet. 1967 May 6;1(7497):980–981. doi: 10.1016/s0140-6736(67)92360-4. [DOI] [PubMed] [Google Scholar]
  10. ENDERS-RUCKLE G. METHODS OF DETERMINING IMMUNITY, DURATION AND CHARACTER OF IMMUNITY RESULTING FROM MEASLES. Arch Gesamte Virusforsch. 1965;16:182–207. doi: 10.1007/BF01253808. [DOI] [PubMed] [Google Scholar]
  11. FOLEY J., WILLIAMS D. Inclusion encephalitis and its relation to subacute sclerosing leucoencephalitis; a report of five cases. Q J Med. 1953 Apr;22(86):157–194. [PubMed] [Google Scholar]
  12. Fraser K. B. Defective and delayed myxovirus infections. Br Med Bull. 1967 May;23(2):178–184. doi: 10.1093/oxfordjournals.bmb.a070541. [DOI] [PubMed] [Google Scholar]
  13. Freeman J. M., Magoffin R. L., Lennette E. H., Herndon R. M. Additional evidence of the relation between subacute inclusion-body encephalitis and measles virus. Lancet. 1967 Jul 15;2(7507):129–131. doi: 10.1016/s0140-6736(67)92965-0. [DOI] [PubMed] [Google Scholar]
  14. Freeman J. M. Treatment of subacute sclerosing panencephalitis with 5-bromo-2-deoxyuridine and pyran copolymer. Neurology. 1968 Jan;18(1 Pt 2):176–192. doi: 10.1212/wnl.18.1_part_2.176. [DOI] [PubMed] [Google Scholar]
  15. GIBBS F. A., GIBBS E. L., CARPENTER P. R., SPIES H. W. Electroencephalographic abnormality in "uncomplicated" childhood diseases. J Am Med Assoc. 1959 Oct 24;171:1050–1055. doi: 10.1001/jama.1959.03010260006002. [DOI] [PubMed] [Google Scholar]
  16. GREENFIELD J. G. Encephalitis and encephalomyelitis in England and Wales during the last decade. Brain. 1950 Jun;73(2):141–166. doi: 10.1093/brain/73.2.141. [DOI] [PubMed] [Google Scholar]
  17. Herndon R. M., Rubinstein L. J. Light and electron microscopy observations on the development of viral particles in the inclusions of Dawson's encephalitis (subacute sclerosing panencephalitis). Neurology. 1968 Jan;18(1 Pt 2):8–20. doi: 10.1212/wnl.18.1_part_2.008. [DOI] [PubMed] [Google Scholar]
  18. Horta-Barbosa L., Fuccillo D. A., Sever J. L., Zeman W. Subacute sclerosing panencephalitis: isolation of measles virus from a brain biopsy. Nature. 1969 Mar 8;221(5184):974–974. doi: 10.1038/221974a0. [DOI] [PubMed] [Google Scholar]
  19. Katz M., Rorke L. B., Masland W. S., Koprowski H., Tucker S. H. Transmission of an encephalitogenic agent from brains of patients with subacute sclerosing panencephalitis to ferrets. Preliminary report. N Engl J Med. 1968 Oct 10;279(15):793–798. doi: 10.1056/NEJM196810102791503. [DOI] [PubMed] [Google Scholar]
  20. Kolár O., Obrucník M., Behounková L., Musil J., Penicková V. Thymectomy in subacute sclerosing leucoencephalitis. Br Med J. 1967 Jul 1;3(5556):22–24. doi: 10.1136/bmj.3.5556.22. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. LORAND B., NAGY T., TARISKA S. Subacute progressive panencephalitis. World Neurol. 1962 May;3:376–394. [PubMed] [Google Scholar]
  22. Ledeen R., Salsman K., Cabrera M. Gangliosides in subacute sclerosing leukoencephalitis: isolation and fatty acid composition of nine fractions. J Lipid Res. 1968 Jan;9(1):129–136. [PubMed] [Google Scholar]
  23. Legg N. J. Virus antibodies in subacute sclerosing panencephalitis: a study of 22 patients. Br Med J. 1967 Aug 5;3(5561):350–352. doi: 10.1136/bmj.3.5561.350. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Lennette E. H., Magoffin R. L., Freeman J. M. Immunologic evidence of measles virus as an etiologic agent in subacute sclerosing panencephalitis. Neurology. 1968 Jan;18(1 Pt 2):21–29. doi: 10.1212/wnl.18.1_part_2.021. [DOI] [PubMed] [Google Scholar]
  25. MALAMUD N., HAYMAKER W., PINKERTON H. Inclusion encephalitis; with a clinicopathologic report of three cases. Am J Pathol. 1950 Jan;26(1):133-53, incl 5 pl. [PMC free article] [PubMed] [Google Scholar]
  26. METZ H., GREGORIOU M., SANDIFER P. SUBACUTE SCLEROSING PAN-ENCEPHALITIS. A REVIEW OF 17 CASES WITH SPECIAL REFERENCE TO CLINICAL DIAGNOSTIC CRITERIA. Arch Dis Child. 1964 Dec;39:554–557. doi: 10.1136/adc.39.208.554. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. OSETOWSKA E., TORCK P. Subacute sclerosing leukoencephalitis. Analysis of complementary features. World Neurol. 1962 Jul-Aug;3:566–579. [PubMed] [Google Scholar]
  28. PAMPIGLIONE G. POLYMYOGRAPHIC STUDIES OF SOME INVOLUNTARY MOVEMENTS IN SUBACUTE SCLEROSING PAN-ENCEPHALITIS. Arch Dis Child. 1964 Dec;39:558–563. doi: 10.1136/adc.39.208.558. [DOI] [PMC free article] [PubMed] [Google Scholar]
  29. PAMPIGLIONE G. PRODROMAL PHASE OF MEASLES: SOME NEUROPHYSIOLOGICAL STUDIES. Br Med J. 1964 Nov 21;2(5420):1296–1300. doi: 10.1136/bmj.2.5420.1296. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Pette E. Measles virus: a causative agent in multiple sclerosis? Neurology. 1968 Jan;18(1 Pt 2):168–169. doi: 10.1212/wnl.18.1_part_2.168. [DOI] [PubMed] [Google Scholar]
  31. Périer O., Vanderhaeghen J. J., Pelc S. Subacute sclerosing leuco-encephalitis. Electron microscopic finding in two cases with inclusion bodies. Acta Neuropathol. 1967 Jul 5;8(4):362–380. doi: 10.1007/BF00696673. [DOI] [PubMed] [Google Scholar]
  32. Rustigian R. Persistent infection of cells in culture by measles virus. I. Development and characteristics of HeLa sublines persistently infected with complete virus. J Bacteriol. 1966 Dec;92(6):1792–1804. doi: 10.1128/jb.92.6.1792-1804.1966. [DOI] [PMC free article] [PubMed] [Google Scholar]
  33. Tellez-Negal I., Harter D. H. Subacute sclerosing leukoencephalitis: ultrastructure of intranuclear and intracytoplasmic inclusions. Science. 1966 Nov 18;154(3751):899–901. doi: 10.1126/science.154.3751.899. [DOI] [PubMed] [Google Scholar]
  34. VAN BOGAERT L. Une leuco-encéphalite sclérosante subaiguë. J Neurol Neurosurg Psychiatry. 1945 Jul-Oct;8:101–120. doi: 10.1136/jnnp.8.3-4.101. [DOI] [PMC free article] [PubMed] [Google Scholar]
  35. Waterson A. P., Almeida J. D. Taxonomic implications of 'myxovirus'. Nature. 1966 Jun 11;210(5041):1138–1140. doi: 10.1038/2101138a0. [DOI] [PubMed] [Google Scholar]
  36. Webb H. E., Smith C. E. Relation of immune response to development of central nervous system lesions in virus infections of man. Br Med J. 1966 Nov 12;2(5523):1179–1181. doi: 10.1136/bmj.2.5523.1179. [DOI] [PMC free article] [PubMed] [Google Scholar]
  37. Zeman W., Kolar O. Reflections on the etiology and pathogenesis of subacute sclerosing panencephalitis. Neurology. 1968 Jan;18(1 Pt 2):1–7. doi: 10.1212/wnl.18.1_part_2.1. [DOI] [PubMed] [Google Scholar]

Articles from Postgraduate Medical Journal are provided here courtesy of BMJ Publishing Group

RESOURCES