Possible treatments for sporadic Creutzfeldt-Jakob disease: a systematic review

Authors

DOI:

https://doi.org/10.11606/issn.1679-9836.v102iespe-203760

Keywords:

Prion disease, Sporadic Creutzfeldt-Jakob disease, Treatment

Abstract

Prion Diseases are rare and fatal diseases that compromises the Nervous System, in which occurs an abnormal accumulation of a protein known as Prion Scrapie (PrPsc). PrPsc is a mutation of a brain tissue protein called Normal Cellular Prion (PrPc) and the accumulation of PrPsc causes spongiform deficiencies. Creutzfeldt-Jakob Disease (CJD) is one of the various Prion Diseases and its sporadic form (sCJD) is the most prevalent. Considering the relentless and rapid progression of the disease, the invariably lethal outcome and the scarcity of therapeutic options for Prion Diseases, especially sCJD, effective treatments become necessary. Thus, the objective of this systematic review is to analyze and identify therapeutic options aimed at sCJD. This research was carried out in the Pubmed and Cochrane databases with the Mesh Terms “Sporadic Creutzfeldt-Jakob disease” and “Treatment”, and the boolean “AND”. Four articles in English were selected, that most aligned ones with the work targets. As the outcome of this research, came to a conclusion that two drugs were well tolerated by the patients, nevertheless they did not deliver a better life quality neither achieved an extended life span. Other drugs are being tested in adult patients, some in clinical trials exhibited good efficacy, minimizing cognitive deficits in CJD patients. However, the studies require a greater number of trials in a wide amount of patients for better conclusion that addresses the treatment of this severe and fatal disease.

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Author Biographies

  • Aline Gabriele Etur dos Santos, Universidade de Mogi das Cruzes. Faculdade de Medicina

    This is a scientific article awarded as Best Work in the Monograph Award category of the XLI University Medical Congress of the Faculty of Medicine of the University of São Paulo (COMU AWARDS 2022) Faculdade de Medicina da Universidade de Mogi das Cruzes - UMC.

  • Gabriel Barbosa Huszcz, Universidade de Mogi das Cruzes. Faculdade de Medicina

    This is a scientific article awarded as Best Work in the Monograph Award category of the XLI University Medical Congress of the Faculty of Medicine of the University of São Paulo (COMU AWARDS 2022) Faculdade de Medicina da Universidade de Mogi das Cruzes - UMC.

  • Henrique Garutti dos Santos, Universidade Nove de Julho. Faculdade de Medicina

    This is a scientific article awarded as Best Work in the Monograph Award category of the XLI University Medical Congress of the Faculty of Medicine of the University of São Paulo (COMU AWARDS 2022) Faculdade de Medicina da UNINOVE.

  • Dyana Alves Henriques, Universidade de Mogi das Cruzes. Faculdade de Medicina

    This is a scientific article awarded as Best Work in the Monograph Award category of the XLI University Medical Congress of the Faculty of Medicine of the University of São Paulo (COMU AWARDS 2022) Professor of Microbiology, Department of Medicine, Faculty of Medicine, University of Mogi das Cruzes

References

Berti V. Príon e doenças priônicas: uma revisão. Colloquium Vitae. 2020;12(2):47-58. doi: https://doi.org/10.5747/cv.2020.v12.n2.v296

Prusiner SB. Príons. PNAS. 1998; 95(23):13363-13383. doi: https://doi.org/10.1073/pnas.95.23.13363

Costa AL, Silva-Junior AC. Prions: uma revisão de suas propriedades bioquímicas e das características patológicas das encefalopatias espongiformes transmissíveis. Rev Arq Cient (IMMES). 2018;1(1):04-13. doi: https://doi.org/10.5935/2595-4407/rac.immes.v1n1p4-13

Machado R, Miranda A, Dantas B. Análise bibliográfica: proteína príon e encefalopatias transmissíveis. Rev Ciên Saúde Nova Esperança. 2020;18(2):88-7. doi: https://doi.org/10.17695/revcsnevol18n2p88-97

Liberati A, Altman DG, Tetzlaff J, Mulrow C, Gøtzsche PC, Ioannidis JP, et al. The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate health care interventions: explanation and elaboration. PLOS Med. 2009;6(7):e1000100. doi: https://doi.org/10.1371/journal.pmed.1000100

Burns PB, Rohrich RJ, Chung KC. The levels of evidence and their role in evidence-based medicine. Plast Reconstr Surg. 2011;128(1):305-310. doi: https://doi.org/10.1097/PRS.0b013e318219c171

Collinge J, Gorham M, Hudson F, Kennedy A, Keogh G, Pal S, et al. Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial. Lancet Neurol. 2009;8(4):334-44. doi: https://doi.org/10.1016/S1474-4422(09)70049-3

Geschwind MD, Kuo AL, Wong KS, Haman A, Devereux G, Raudabaugh BJ, et al. Quinacrine treatment trial for sporadic Creutzfeldt-Jakob disease. Neurology. 2013;81(23):2015-23. doi: https://doi.org/10.1212/WNL.0b13e3182a9f3b4

Haïk S, Marcon G, Mallet A, Tettamanti M, Welaratne A, Giaccone G, et al. Doxycycline in Creutzfeldt-Jakob disease: a phase 2, randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2014;13(2):150-8. doi: https://doi.org/10.1016/S1474-4422(13)70307-7

Varges D, Manthey H, Heinemann U, Ponto C, Schmitz M, Schulz-Schaeffer WJ, et al. Doxycycline in early CJD: a double-blinded randomised phase II and observational study. J Neurol Neurosurg Psychiatry. 2017 Feb;88(2):119-125. doi: http://dx.doi.org/10.1136/jnnp-2016-313541

Mahat S, Asuncion RMD. Kuru. [Updated 2022 Jun 5]. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK559103/

Duncan BB, Schmidt MI, Giugliani ERJ. Medicina ambulatorial: condutas de atenção primária baseada em evidências. 3a ed. Porto Alegre: Artmed Editora; 2004.

Otto M, Cepek L, Ratzka P, Doehlinger S, Boekhoff I, Wiltfang J, et al. Efficacy of flupirtine on cognitive function in patients with CJD: A double-blind study. Neurology. 2004;62(5):714-8. doi: https://doi.org/10.1212/01.WNL.0000113764.35026.EF

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Published

2023-02-06

How to Cite

Santos, A. G. E. dos, Huszcz, G. B., Santos, H. G. dos, & Henriques, D. A. . (2023). Possible treatments for sporadic Creutzfeldt-Jakob disease: a systematic review. Revista De Medicina, 102(esp), e-203760. https://doi.org/10.11606/issn.1679-9836.v102iespe-203760