Treatment with Anti-malaria Agents, Quinacrine and Quinine, for Creutzfeldt - Jaicob disease patients
Yoshio Tsuboi ^ Fujio Fujiki \ Atsushi Yamauchi ^, Katsumi Doh-ura^
Yasufumi Kataoka^ and Tatsuo Yamada ^
^Neurology, FukuokaUniversity, 7-45-1 Nanakuma, Jonan-ku, Fukuoka 814-0180 Japan ^ Pharmacology, Fukuoka University ^ Prion Researc Tohoku University <e-mail> tsuboi@cis.fukuoka-u.ac.jp
Abstract
Objects
To assess effects and safety of treatment w^ith anti-malaria agents as treat- ment for Creutzfeldt-Jakob disease (CJD).
Background
There have been no effective treatments for CJD. Several chemicals in- hibit an accumulation or a conformational change of prion protein in vitro.
Anti-malaria agents such as quinacrine and quinine are know^n to have anti-prion effects.
Materials and Methods
The study WSLS approved by the institutional ethics committee, and pa- tients' relatives gave consent for the procedure. Thirty-one cases WQTQ
treated w^ith quinacrine including 22 with sporadic CJD, 5 cases w^ith iatrogenic CJD, and 4 cases w^ith familial CJD. Quinacrine 300 mg/day w^as administered enterally for three months. Six cases w^ith sporadic CJD treated w^ith quinine. Quinine 1.5 g/day w^as administrated enterally for three months. Motor and cognitive functions w^ere monitored.
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Results
Anti-malaria agents were well tolerated except for abnormal liver function tests, dermatitis or thrombocytopenia. Increased arousal levels and re- sponses to visual and auditory stimulation were seen in 12 patients with quinacrine treatment. Out of 22 sporadic CJD, only one case improved from akinetic mutism. The other 8 patients, who had positive responses as eye contact to verbal and/or visual stimuli before treatment, showed clinical improvement. Out of 5 patients treated with quinine, two cases showed improved response to auditory and visual stimuli. Clinical im- provement was transient and lasted for one to two months during treat- ment.
Conclusion
This is the first observation of moderate reversibility of cognitive function in a large series of patients with CJD treated with anti-malaria agents.
Liver dysfunction is the major complication and sometimes requires dis- continuing this medication. It is also suggested that quinacrine/quinine could be the treatment of choice for patients with CJD. However, these agents are not able to prevent the clinical declines or to improve these fea- tures. Optimal treatment regimen and further clinical trial will be re- quired.
