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CJD without borders

Nature Reviews Neurology volume 17, page 723 (2021)Cite this article

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In their recent Review, Watson et al. discussed the need for ongoing international surveillance of Creutzfeldt–Jakob Disease (CJD) (Watson, N. et al. The importance of ongoing international surveillance for Creutzfeldt–Jakob disease. Nat. Rev. Neurol. 17, 362–379 (2021)1). CJD belongs to a unique group of rare neurodegenerative pathologies called prion diseases for which, to date, no cure or effective treatment is available. Despite being rare, CJD is a great concern to public health, particularly its variant (vCJD) and iatrogenic (iCJD) forms, which have a long incubation period2.

We agree with Watson et al.1 that reference centres and international collaboration are crucial for improved management and discussion of rare diseases such as CJD. This kind of collaboration is pivotal to help countries with a deficient or nonexistent surveillance system to manage cases with greater accuracy and efficiency. For example, in Brazil, a collaboration between the Ministry of Health and several research groups was established in the early 2000s and resulted in a protocol for notification, follow-up and management of reported cases of CJD that is still in use today3. However, in 2018, some of these collaborations were discontinued and since then genetic assessment has not been performed in individuals with suspected CJD, worsening an already weak surveillance system4. Although the Brazilian CJD protocol involves a simple but well-designed report sheet, accessing the resulting epidemiological data is not straightforward. The data must be requested from the Brazilian Ministry of Health online. When provided, the data are codified in almost all fields. When we compared data obtained via this route with a Ministry of Health publication covering the same time period5, we identified some differences in the number of cases reported, highlighting the challenges involved in collecting and sharing accurate data on rare diseases.

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References

  1. Watson, N. et al. The importance of ongoing international surveillance for Creutzfeldt–Jakob disease. Nat. Rev. Neurol. 17, 362–379 (2021).

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  5. Situação epidemiológica dos casos da doença de Creutzfeldt-Jakob, Brasil, 2005 a 2013 [Portuguese]. Secr. Vigilância em Saúde − Ministério da Saúde 2, 35–42 (2016).

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Acknowledgements

The authors acknowledge Terri Lindsay from National CJD Research & Surveillance Unit (United Kingdom) for contributing to CJD without Borders Web App and the Brazilian Ministry of Health for providing the data for the DCJ EpiShiny Web App. They also thank Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) and Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) for the financial support.

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Authors and Affiliations

  1. Laboratório de Imunopatologia Keizo Asami, Universidade Federal de Pernambuco, Recife, Pernambuco, Brazil

    José Eriton G. Cunha & João R. M. Oliveira

  2. Instituto Aggeu Magalhães, Fundação Oswaldo Cruz, Recife, Pernambuco, Brazil

    Jessica C. F. Nascimento

  3. Neuropsychiatry Department, Universidade Federal de Pernambuco, Recife, Pernambuco, Brazil

    João R. M. Oliveira

Authors
  1. José Eriton G. Cunha
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  2. Jessica C. F. Nascimento
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  3. João R. M. Oliveira
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Corresponding author

Correspondence to José Eriton G. Cunha.

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The authors declare no competing interests.

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Related links

CJD International Surveillance Network (CJDISN): https://www.eurocjd.ed.ac.uk/

CJD Without Borders: https://epicjd.shinyapps.io/cjdMAP/

DCJ Epishiny: https://epicjd.shinyapps.io/dcjBRASIL/

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Cunha, J.E.G., Nascimento, J.C.F. & Oliveira, J.R.M. CJD without borders. Nat Rev Neurol 17, 723 (2021). https://doi.org/10.1038/s41582-021-00566-w

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