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Systematic review of pharmacological management in Creutzfeldt-Jakob disease: no options so far?

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posted on 2022-11-22, 07:38 authored by Luiz Henrique Lélis Miranda, André Felipe Pastick de Holanda Oliveira, Davi Mariano de Carvalho, Gustavo Marques Figueredo Souza, João Guilherme Monteiro Magalhães, Joselmo Alberto Cabral Júnior, Pedro Thadeu Mertens Brainer de Queiroz Lima, Renato Melo Aguiar Júnior, Sérgio Pereira Lins Filho, Hugo Moura de Albuquerque Melo

Abstract Background The Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy that manifests as a rapidly progressive dementia syndrome. Currently, CJD has no cure, and many patients die within the first year, but some drugs are being studied as options for managing this condition. Objective To evaluate the effectiveness of pharmacological treatments offered to patients with CJD as a means to increase survival and reduce cognitive deterioration. Methods A systematic review of the literature was performed using 4 independent reviewers and 1 extra reviewer to resolve possible divergences in the search and analysis of papers indexed in MedLINE (PubMed), SciELO and Lilacs databases. The Medical Subject Heading (MeSH) terms used were: prion diseases, Creutzfeldt-Jakob disease, pharmacologic therapy, therapeutics, quinacrine, doxycycline, flupirtine, and pentosan polysulfate, with the Boolean operators AND and OR. This search included controlled clinical trials, uncontrolled clinical trials, and case series published from the year 2000 onwards, in the English language. Results A total of 85 papers were found using the descriptors used. At the end of the selection analyses, 9 articles remained, which were analyzed fully and individually. Conclusions None of the drugs evaluated proved significantly effective in increasing survival in patients with CJD. Flupirtine appears to have a beneficial effect in reducing cognitive deterioration in patients with CJD. However, additional studies are needed to establish better evidence and therapeutic options for the management of patients with CJD.