Rinsho Shinkeigaku (Clinical Neurology)

The 50th Annual Meeting of the Japanese Society of Neurology

Innovation of therapeutics and prophylaxis for prion diseases

Katsumi Doh-ura, M.D.

Division of Prion Biology, Tohoku University Graduate School of Medicine

There is no established treatment for prion diseases; however, recently several drug candidates, including pentosan polysulfate and doxycycline, have been clinically used on a trial basis to prevent accumulation of abnormal prion protein in the brain. So far the outcome of the trials is still very far from the goal where a complete cure of the diseases is expected. In order to bridge the gap between the reality and the ideal, the followings are suggested. First, combination therapy needs to be developed against multi-targets: inhibition of prion replication; degradation and scavengery of prion; inhibition of prion-related neurodegeneration. Secondly, preclinical diagnostic means, by which healthy prion-carriers can be revealed before the onset of the diseases, should be explored for earlier therapeutic interventions. The last is to disclose intrinsic disease susceptibility factors and environmental factors, both of which could solely or jointly facilitate in suppressing prion replication and disease progress. Exploitation of these items should be tough but will be deserved for overcoming the fatal diseases.
Full Text of this Article in Japanese PDF (206K)

(CLINICA NEUROL, 49: 946|948, 2009)
key words: therapeutics, prion disease, combination therapy, preclinical diagnosis, disease susceptibility

(Received: 22-May-09)