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

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.

Creutzfeldt-Jakob-Krankheit – eine mögliche Differentialdiagnose an einer Psychiatrie

“…Es gibt bisher noch keine medikamentöse Therapie, die mit einer signifikant längeren Überlebensrate oder signifikanten Verminderung der neurologischen und psychiatrischen Symptomatik einhergeht. Die durchgeführtenStudiensind durch kleine Proband*innengruppen und teils unscharfe Behandlungszeitträume limitiert, häufig finden sich in der Literatur nicht randomisierte Studien und Fallberichte [20]. Unter den pharmakologischen Therapieversuchen finden sich Flupirtinmaleat, ein Nicht-Opioid-Analgetikum, Pentosan [21], Polysulfate, ein synthetisiertes Heparinoid, welches intraventrikulär verabreicht wird, Quinacrine [22], ein Anti-Malaria-Therapeutikum, Chlorpromazin, ein Antipsychotikum, und das Tetrazyklinantibiotikum Doxycyklin, welches in vitro und in Tierstudien die abnorme Faltung der Proteine verhindert [23].…”

Doença de Creutzfeldt-Jakob: um relato de caso da forma esporádica

“…25 Em contrapartida, os recentes avanços em relação à compreensão da patogênese da DCJ, aumentaram a expectativa pelo surgimento de intervenções terapêuticas eficazes. 26 Neste contexto, a abordagem com o uso do medicamento antipsicótico Clorpromazina vem sendo utilizada visando a eliminação de príons, com bons resultados em camundongos até o momento, mas ainda sem expectativa de ser testada em seres humanos. 27,28 Desse modo, o tratamento atual da DCJ é apenas de suporte e seu desfecho é fatal, sendo que a maioria dos doentes evolui para óbito em até um ano do início dos sintomas.…”