Skip to main content
PMC home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1972 Feb;35(1):1–10. doi: 10.1136/jnnp.35.1.1

Subacute spongiform encephalopathy and its relation to Jakob-Creutzfeldt disease: report on six cases

Y Goldhammer 1,2,3,4, J J Bubis 1,2,3,4, Ida Sarova-Pinhas 1,2,3,4, J Braham 1,2,3,4
PMCID: PMC493954  PMID: 4554588

Abstract

An account is given of six cases of Jakob-Creutzfeldt disease confirmed at necropsy. The rapid fatal outcome within three months was typical of the variety designated subacute spongiform encephalopathy (Nevin-Jones). The characteristic EEG changes were found to be of great value in making an early diagnosis and, together with akinetic mutism and myoclonus, constituted an easily recognizable picture. Treatment with idoxyuridine was ineffective in one case in which electronmicroscopy of brain biopsy material supported the diagnosis.

Full text

PDF
10

Images in this article

3Image
on p.3
3Image
on p.3
4Image
on p.4
5Image
on p.5

Selected References

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

  1. BROWNELL B., OPPENHEIMER D. R. AN ATAXIC FORM OF SUBACUTE PRESENILE POLIOENCEPHALOPATHY (CREUTZFELDT-JAKOB DISEASE). J Neurol Neurosurg Psychiatry. 1965 Aug;28:350–361. doi: 10.1136/jnnp.28.4.350. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Beck E., Daniel P. M., Matthews W. B., Stevens D. L., Alpers M. P., Asher D. M., Gajdusek D. C., Gibbs C. J., Jr Creutzfeldt-Jakob disease. The neuropathology of a transmission experiment. Brain. 1969;92(4):699–716. doi: 10.1093/brain/92.4.699. [DOI] [PubMed] [Google Scholar]
  3. Behar M., Sroka C., Elian M., Kott E., Korczyn A., Bornstein B., Sandbank U. [Creutzfeld-Jakob disease and its relation to pre-senile dementia]. Harefuah. 1969 Oct 1;77(7):275–279. [PubMed] [Google Scholar]
  4. Braham J. Jakob-Creutzfeldt disease: treatment by amantadine. Br Med J. 1971 Oct 23;4(5781):212–213. doi: 10.1136/bmj.4.5781.212. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Brion S. Encéphalopathie spongieuse de la présénilité et syndrome de Creutzfeldt-Jakob. Acta Neuropathol. 1967;(Suppl):16–21. [PubMed] [Google Scholar]
  6. CHRISTENSEN E., BRUN A. Subacute spongiform encephalopathy. Spongiform cerebral atrophy. Neurology. 1963 Jun;13:455–463. doi: 10.1212/wnl.13.6.455. [DOI] [PubMed] [Google Scholar]
  7. Foncin J. F. Etude ultrastructurale de la maladie de Creutzfeldt-Jakob. Acta Neuropathol. 1967;(Suppl):127–130. [PubMed] [Google Scholar]
  8. Freeman J. M. Treatment of Dawson's encephalitis wit5-bromo-2'-deoxyuridine. Double-blind study. Arch Neurol. 1969 Oct;21(4):431–434. doi: 10.1001/archneur.1969.00480160103012. [DOI] [PubMed] [Google Scholar]
  9. Gajdusek D. C., Gibbs C. J., Alpers M. Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature. 1966 Feb 19;209(5025):794–796. doi: 10.1038/209794a0. [DOI] [PubMed] [Google Scholar]
  10. Gajdusek D. C., Gibbs C. J., Jr, Alpers M. Transmission and passage of experimenal "kuru" to chimpanzees. Science. 1967 Jan 13;155(3759):212–214. [PubMed] [Google Scholar]
  11. Gibbs C. J., Jr, Gajdusek D. C., Asher D. M., Alpers M. P., Beck E., Daniel P. M., Matthews W. B. Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to the chimpanzee. Science. 1968 Jul 26;161(3839):388–389. doi: 10.1126/science.161.3839.388. [DOI] [PubMed] [Google Scholar]
  12. Gibbs C. J., Jr, Gajdusek D. C. Infection as the etiology of spongiform encephalopathy (Creutzfeldt-Jakob disease). Science. 1969 Sep 5;165(3897):1023–1025. doi: 10.1126/science.165.3897.1023. [DOI] [PubMed] [Google Scholar]
  13. JONES D. P., NEVIN S. Rapidly progressive cerebral degeneration (subacute vascular encephalopathy) with mental disorder, focal disturbances, and myoclonic epilepsy. J Neurol Neurosurg Psychiatry. 1954 May;17(2):148–159. doi: 10.1136/jnnp.17.2.148. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Jacob H., Van Bogaert L. Symposium concernant les dégénérescences spongieuses de la présénilité. Synthèse des apports histopathologiques. Acta Neuropathol. 1967;(Suppl):148–151. [PubMed] [Google Scholar]
  15. KATZMAN R., KAGAN E. H., ZIMMERMAN H. M. A case of Jakob-Creutzfeldt disease. I. Clinicopathological analysis. J Neuropathol Exp Neurol. 1961 Jan;20:78–94. doi: 10.1097/00005072-196101000-00004. [DOI] [PubMed] [Google Scholar]
  16. Kidd M. Some electron microscopical observations on status spongiosus. Acta Neuropathol. 1967;(Suppl):137–144. doi: 10.1007/978-3-642-48255-7_27. [DOI] [PubMed] [Google Scholar]
  17. Lafon R., Labauge R., Van Bogaert L., Castan P. Sur l'unité histopathologique des encéphalopathies subaiguës (types Creutzfeldt-Jakob, Heidenhain et Nevin) Rev Neurol (Paris) 1965 Mar;112(3):201–227. [PubMed] [Google Scholar]
  18. Liversedge L. A., Swinburn W. R., Yuill G. M. Idoxuridine and motor neurone disease. Br Med J. 1970 Mar 21;1(5698):755–756. doi: 10.1136/bmj.1.5698.755-d. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Macchi G., Lechi A. L'état spongieux dans le diagnostic histopathologique des encéphalopathies du présénium. Acta Neuropathol. 1967;(Suppl):54–55. [PubMed] [Google Scholar]
  20. May W. W. Creutzfeldt-Jakob disease. 1. Survey of the literature and clinical diagnosis. Acta Neurol Scand. 1968;44(1):1–32. doi: 10.1111/j.1600-0404.1968.tb07440.x. [DOI] [PubMed] [Google Scholar]
  21. NEVIN S., McMENEMEY W. H., BEHRMAN S., JONES D. P. Subacute spongiform encephalopathy--a subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy). Brain. 1960 Dec;83:519–564. doi: 10.1093/brain/83.4.519. [DOI] [PubMed] [Google Scholar]
  22. Nevin S. On some aspects of cerebral degeneration in later life. Proc R Soc Med. 1967 Jun;60(6):517–526. [PMC free article] [PubMed] [Google Scholar]
  23. PALLIS C. A., SPILLANE J. D. A subacute progressive encephalopathy with mutism, hypokinesia, rigidity, and myoclonus. Q J Med. 1957 Jul;26(103):349–373. [PubMed] [Google Scholar]
  24. Robinson N. Creutzfeldt-Jakob's disease: a histochemical study. Brain. 1969;92(3):581–588. doi: 10.1093/brain/92.3.581. [DOI] [PubMed] [Google Scholar]
  25. SIEDLER H., MALAMUD N. CREUTZFELDT-JAKOB'S DISEASE. CLINICOPATHOLOGIC REPORT OF 15 CASES AND REVIEW OF THE LITERATURE (WITH SPECIAL REFERENCE TO A RELATED DISORDER DESIGNATED AS SUBACUTE SPONGIFORM ENCEPHALOPATHY). J Neuropathol Exp Neurol. 1963 Jul;22:381–402. [PubMed] [Google Scholar]
  26. Terzian H., Rizzuto N., Patarnello L., Martin J. J. Sur une forme particuliére de la maladie de Creutzfeldt-Jakob, avec dissociation anatomo-clinique. Acta Neuropathol. 1967;(Suppl):37–41. [PubMed] [Google Scholar]
  27. Vernon M. L., Horta-Barbosa L., Fuccillo D. A., Sever J. L., Baringer J. R., Birnbaum G. Virus-like particles and nucleoprotein-type filaments in brain tissue from two patients with Creutzfeldt-Jakob disease. Lancet. 1970 May 9;1(7654):964–966. doi: 10.1016/s0140-6736(70)91095-0. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES