Home LiteratureArticle Details
PMID: 4554588 Published · ppublish English Journal Article

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

Journal of neurology, neurosurgery, and psychiatry ·Vol. 35 ·No. 1 ·1972-02-00 ·Pages 1-10

Goldhammer Y, Bubis JJ, Sarova-Pinhas I, Braham J

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.

MeSH Terms
Adult Aged Brain Diseases Central Nervous System Diseases Cerebral Arteries/pathology Cerebral Cortex/pathology Creutzfeldt-Jakob Syndrome/diagnosis,drug therapy,pathology Electroencephalography Encephalitis/complications Extremities Female Humans Idoxuridine/therapeutic use Male Middle Aged Muscular Diseases Slow Virus Diseases/complications
Chemicals
Idoxuridine
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Goldhammer Y
Bubis J J
Sarova-Pinhas I
Braham J
References (27)
27 references, click to expand
  1. Rapidly progressive cerebral degeneration (subacute vascular encephalopathy) with mental disorder, focal disturbances, and myoclonic epilepsy.
    J Neurol Neurosurg Psychiatry. 1954 May;17(2):148-59 PMID: 13163708
  2. [Symposium on presenile spongy encephalopathies. Synthesis of histopathologic contributions].
    Acta Neuropathol. 1967;:Suppl 3:148-51 PMID: 6074640
  3. Creutzfeldt-Jakob disease. The neuropathology of a transmission experiment.
    Brain. 1969;92(4):699-716 PMID: 4903507
  4. Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to the chimpanzee.
    Science. 1968 Jul 26;161(3839):388-9 PMID: 5661299
  5. [Spongy encephalopathy of presenility and Creutzfeldt-Jakob syndrome].
    Acta Neuropathol. 1967;:Suppl 3:16-21 PMID: 6074643
  6. Some electron microscopical observations on status spongiosus.
    Acta Neuropathol. 1967;:Suppl 3:137-44 PMID: 6074638
  7. [On a peculiar form of Creutzfeldt-Jakob disease, with anatomo-clinical dissociation].
    Acta Neuropathol. 1967;:Suppl 3:37-41 PMID: 6074647
  8. Experimental transmission of a Kuru-like syndrome to chimpanzees.
    Nature. 1966 Feb 19;209(5025):794-6 PMID: 5922150
  9. Infection as the etiology of spongiform encephalopathy (Creutzfeldt-Jakob disease).
    Science. 1969 Sep 5;165(3897):1023-5 PMID: 5804726
  10. 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 PMID: 14045000
  11. Transmission and passage of experimenal "kuru" to chimpanzees.
    Science. 1967 Jan 13;155(3759):212-4 PMID: 6015529
  12. Creutzfeldt-Jakob's disease: a histochemical study.
    Brain. 1969;92(3):581-8 PMID: 4308805
  13. [Creutzfeld-Jakob disease and its relation to pre-senile dementia].
    Harefuah. 1969 Oct 1;77(7):275-9 PMID: 4920489
  14. Virus-like particles and nucleoprotein-type filaments in brain tissue from two patients with Creutzfeldt-Jakob disease.
    Lancet. 1970 May 9;1(7654):964-6 PMID: 4191933
  15. Jakob-Creutzfeldt disease: treatment by amantadine.
    Br Med J. 1971 Oct 23;4(5781):212-3 PMID: 4940159
  16. [Ultrastructural study of Creutzfeldt-Jakob disease].
    Acta Neuropathol. 1967;:Suppl 3:127-30 PMID: 6074636
  17. A case of Jakob-Creutzfeldt disease. I. Clinicopathological analysis.
    J Neuropathol Exp Neurol. 1961 Jan;20:78-94 PMID: 13751839
  18. AN ATAXIC FORM OF SUBACUTE PRESENILE POLIOENCEPHALOPATHY (CREUTZFELDT-JAKOB DISEASE).
    J Neurol Neurosurg Psychiatry. 1965 Aug;28:350-61 PMID: 14338125
  19. Creutzfeldt-Jakob disease. 1. Survey of the literature and clinical diagnosis.
    Acta Neurol Scand. 1968;44(1):1-32 PMID: 4884376
  20. [On the histopathological oneness of subacute encephalopathies (Creutzfeldt-Jakob, Heidenhain and Nevin types)].
    Rev Neurol (Paris). 1965 Mar;112(3):201-27 PMID: 5854461
  21. On some aspects of cerebral degeneration in later life.
    Proc R Soc Med. 1967 Jun;60(6):517-26 PMID: 20918952
  22. Treatment of Dawson's encephalitis wit5-bromo-2'-deoxyuridine. Double-blind study.
    Arch Neurol. 1969 Oct;21(4):431-4 PMID: 4186002
  23. Idoxuridine and motor neurone disease.
    Br Med J. 1970 Mar 21;1(5698):755-6 PMID: 5440560
  24. [The spongy state in the histopathological diagnosis of encephalopathies of presenility].
    Acta Neuropathol. 1967;:Suppl 3:54-5 PMID: 6074650
  25. Subacute spongiform encephalopathy--a subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy).
    Brain. 1960 Dec;83:519-64 PMID: 13728615
  26. A subacute progressive encephalopathy with mutism, hypokinesia, rigidity, and myoclonus.
    Q J Med. 1957 Jul;26(103):349-73 PMID: 13466002
  27. Subacute spongiform encephalopathy. Spongiform cerebral atrophy.
    Neurology. 1963 Jun;13:455-63 PMID: 14021083
Article Info
Journal
Journal of neurology, neurosurgery, and psychiatry
Abbr.
J Neurol Neurosurg Psychiatry
ISSN
0022-3050
DOI
10.1136/jnnp.35.1.1
Published
1972-02-00
Pages
1-10
Language
English
Region
England
NLM ID
2985191R
PMCID
PMC493954
Subset
IM
External Links
PubMed source DOI: 10.1136/jnnp.35.1.1 PMC Full Text View journal details
Analysis Services
Analysis Services

Contact

No. 2 Wenbo Road, Zhangqiu District, Jinan, Shandong

Qilu Normal University · Genelibs Bioinformatics Lab

750 Shunhua Rd, Jinan

2F, Bldg F, University Science Park

Tel: 0531-88819269

WeChat Official Account

Follow our WeChat subscription account for real-time updates and the latest in medical and biological research.


Business Email

E-mail: product@genelibs.com

Copyright © 2026.Genelibs Jinan Honghui Biotechnology Co., Ltd. 鲁ICP备20007159号-2