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

• Published in: Arquivos de neuro-psiquiatria Vol. 80; no. 8; pp. 837 - 844
• Main Authors: Miranda, Luiz Henrique Lélis, Oliveira, André Felipe Pastick de Holanda, Carvalho, Davi Mariano de, Souza, Gustavo Marques Figueredo, Magalhães, João Guilherme Monteiro, Júnior, Joselmo Alberto Cabral, Lima, Pedro Thadeu Mertens Brainer de Queiroz, Júnior, Renato Melo Aguiar, Filho, Sérgio Pereira Lins, Melo, Hugo Moura de Albuquerque
• Format: Journal Article
• Language: English; Portuguese
• Published: Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil Thieme Revinter Publicações Ltda 01.08.2022; Arquivos de Neuro-Psiquiatria; Academia Brasileira de Neurologia - ABNEURO; Academia Brasileira de Neurologia (ABNEURO)
• Subjects: Aminopyridines; Clinical trials; Cognitive ability; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome; Creutzfeldt-Jakob Syndrome - drug therapy; Creutzfeldt-Jakob Syndrome - psychology; Dementia disorders; Divergence; Doenças Priônicas; Doxycycline; Doxycycline - therapeutic use; Drug development; Drug therapy; Encephalopathy; Humans; Literature reviews; NEUROSCIENCES; Patients; Pentosan polysulfate; Pentosan Sulfuric Polyester - therapeutic use; Prion Diseases; PSYCHIATRY; Quinacrine; Quinacrine - therapeutic use; Spongiform encephalopathy; Systematic review; Síndrome de Creutzfeldt-Jakob; Terapêutica; Therapeutics; View and Review; Terapêutica; Prion Diseases; Síndrome de Creutzfeldt-Jakob; Creutzfeldt-Jakob Syndrome; Therapeutics; Doenças Priônicas
• ISSN: 0004-282X; 1678-4227; 1678-4227
• DOI: 10.1055/s-0042-1755341

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.