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Treating Prion Disorders

Posted by steelgraham on 03 Apr 2008 at 15:54 GMT


Dear Authors,

This is an interesting Paper that has just come to my attention.

I was unaware of the small group of CJD patients who have received compassionated treatment with daily doses of 100 mg/kg doxycycline and clearly be interest in reading the forthcoming Manuscript by Tagliavini et al in this regard.


"Currently, there is no therapy for prion diseases in humans. Three drugs, pentosan polysulfate [8], [9], quinacrine [10], [11] and flupirtrine [12], are currently used in the clinical management of human prion diseases, although none of them have yet been proved to be effective, reinforcing the importance of identifying new treatment strategies."

Are the Authors aware of a Paper by Bone et al published last month in the European Journal of Neurology, PMID: 18355301, "Intraventricular pentosan polysulphate in human prion diseases: an observational study in the UK." and do they have any comment with regards to the following from that Paper?

"....Of the four vCJD patients, in whom untreated comparison patients are more contemporaneous, one survived for 16 months (beyond the median of 13 months in (M. Pocchiari, personal communication) and 14 months in [21]) and three are still alive in August 2007 (all exceeding the mean, median and range of untreated patients in both natural history studies). The probability of this occurring (if there were no difference between the treated and natural history patients and no other potential biases) is 0.01, indicating some suggestion/ possibility of an effect."


It is also worth pointing out that without a very early diagnosis, one has to be realistic as to what one may expect from therapeutic intervention.

Kind regards,

Graham Steel
Co-founder CJD Alliance