Rinsho Shinkeigaku (Clinical Neurology)

The 45th Annual Meeting of the Japanese Society of Neurology

Prion diseases: disease diversity and therapeutics

Katsumi Doh-ura, M.D.

Department of Prion Research, Tohoku University Graduate School of Medicine

More than one hundred victims of iatrogenic CJD with cadaveric dura mater grafting have been recognized in Japan, and the people have been also exposed to a risk of outbreaks of variant CJD. These diseases are distinct from other forms of prion diseases as well as other neuropsychiatric disorders, but on an early clinical stage, their differential diagnoses from other atypical forms of prion diseases are not necessarily easy. Thus, atypical forms of prion diseases were overviewed and discussed here. In addition, data on recent clinical trials of enteral anti-malarial drug (quinacrine or quinine) treatment or intracerebroventricular pentosan polysulfate treatment were presented and discussed, because research progress in the therapeutics for prion diseases has been remarkably made on the basis of the prevalence of those acquired forms of prion diseases.

(CLINICA NEUROL, 44: 855|856, 2004)
key words: variant CJD, differential diagnosis, therapeutics, antimalarial, pentosan polysulfate

(Received: 13-May-04)