Table 1.

Recent experimental and translational studies of Creutzfeldt–Jakob disease (CJD) patients

Country	Compound	Study period	Report	Study type	Result	Note
Germany	Flupirtine	1997–2001	Otto et al. 2004	Randomized, double-blind trial; 28 patients of CJD	Effective in cognitive functions but ineffective in survival	No preclinical studies of the drug in prion-infected cells or animals
Japan	PPS	2004–2007	Tsuboi et al. 2009	Open prospective observation; 11 patients of CJD	No apparent improvement of clinical features	Continuous cerebroventricular infusion of the compound through an osmotic pump device
United Kingdom		2003–?	Newman et al. 2014, and others	Case reports (unorganized trial); five patients of variant CJD	Significantly extended survival in four patients
United Kingdom	Quinacrine	2004–2007	Collinge et al. 2009	Open-labeled, patient-preference trial (PRION-1); 107 patients of CJD	No difference in mortality between treated and nontreated groups	Transient improvement of mental and neurological signs caused by adverse brain reactions to the drug
United States		2005–2009	Geschwind et al. 2013	Double-blind, placebo-controlled, stratified randomization trial; 54 patients of CJD	No significant difference between drug groups and placebo groups in survival
Italy	Doxycycline	2007–2010	Haïk et al. 2014	Double-blind, placebo-controlled, randomized trial; 121 patients of CJD	No significant difference between drug groups and placebo groups in survival	Minimal benefits in prion-infected animals even in cerebroventricular liposomal delivery of the drug
France		2009–2012

PPS, Pentosane polysulfate.