Skip to main content
SpringerLink
Log in

Early Cortical Diffusion Restriction in Creutzfeldt-Jakob Disease: a Case Report

  • Case Report
  • Published:
SN Comprehensive Clinical Medicine Aims and scope Submit manuscript

Abstract

Creutzfeldt-Jakob disease is a spongiform encephalopathy with rapidly progressing neurological symptoms. Always is there a fatal outcome, usually within 1 year of symptom onset. Cortical diffusion restriction is an early diagnostic finding in the disease. In a patient with multiple sclerosis, we found cortical diffusion restriction 27 months prior to symptom onset. It is the second report of the existence of such a long preclinical MRI-positive phase in sCJD. This case report might change the perspective on a generally accepted extremely rapid progressive disease and could even imply a window of opportunity for possible future treatment.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

Data Availability

Not applicable.

Code Availability

Not applicable.

References

  1. Ladogana A, Puopolo M, Croes EA, Budka H, Jarius C, Collins S, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology. 2005;64(9):1586–91.

    Article  CAS  PubMed  Google Scholar 

  2. Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann U, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain. 2009;132(Pt 10):2659–68.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Uttley L, Carroll C, Wong R, Hilton DA, Stevenson M. Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation. Lancet Infect Dis. 2020;20(1):e2–10.

    Article  PubMed  Google Scholar 

  4. Hermann P, Appleby B, Brandel JP, Caughey B, Collins S, Geschwind MD, et al. Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease. Lancet Neurol. 2021;20(3):235–46.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Shiga Y, Miyazawa K, Sato S, Fukushima R, Shibuya S, Sato Y, et al. Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology. 2004;63(3):443–9.

    Article  CAS  PubMed  Google Scholar 

  6. Alvarez FJ, Bisbe J, Bisbe V, Dávalos A. Magnetic resonance imaging findings in pre-clinical Creutzfeldt-Jakob disease. Int J Neurosci. 2005;115(8):1219–25.

    Article  CAS  PubMed  Google Scholar 

  7. Zanusso G, Camporese G, Ferrari S, Santelli L, Bongianni M, Fiorini M, et al. Long-term preclinical magnetic resonance imaging alterations in sporadic Creutzfeldt-Jakob disease. Ann Neurol. 2016;80(4):629–32.

    Article  PubMed  Google Scholar 

  8. Koizumi R, Ueda N, Mugita A, Kimura K, Kishida H, Tanaka F. Case report: extremely early detection of preclinical magnetic resonance imaging abnormality in Creutzfeldt-Jakob disease with the V180I mutation. Front Neurol. 2021;12: 751750.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Yasuda M, Sugiyama A, Hokkoku H, Suichi T, Ito K, Satoh K, et al. Propagation of diffusion-weighted MRI abnormalities in the preclinical stage of sporadic Creutzfeldt-Jakob disease. Neurology. 2022;99(16):699–702.

    Article  CAS  PubMed  Google Scholar 

Download references

Funding

Not applicable.

Author information

Authors and Affiliations

  1. Department of Neurology, Onze-Lieve-Vrouw Hospital, Aalst, Belgium

    Arne Hostens, Lucas Dekesel, Gert Cypers & Jan Vynckier

  2. Department of Neurology, Brussels University Hospital, Brussels, Belgium

    Arne Hostens

  3. Department of Radiology and Medical Imaging, Ghent University Hospital, Ghent, Belgium

    Lucas Dekesel

Authors
  1. Arne Hostens
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lucas Dekesel
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Gert Cypers
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jan Vynckier
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

JV and GC contributed to the conception and design of the study. AH and LD wrote the first draft of the manuscript. All authors contributed to data analysis, data interpretation, and manuscript revision.

Corresponding author

Correspondence to Jan Vynckier.

Ethics declarations

Ethics Approval

Not applicable.

Consent to Participate

Not applicable.

Consent for Publication

Written informed patient consent for publication of this case report has been obtained.

Conflict of Interest

The authors declare no competing interests.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Medicine

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hostens, A., Dekesel, L., Cypers, G. et al. Early Cortical Diffusion Restriction in Creutzfeldt-Jakob Disease: a Case Report. SN Compr. Clin. Med. 5, 274 (2023). https://doi.org/10.1007/s42399-023-01606-w

Download citation

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s42399-023-01606-w

Keywords

Search

Navigation