Prognosis and treatment

All forms of prion diseases that are currently recognized are invariably fatal and follow a relentlessly progressive course. No currently available treatment alters the clinical course of the disease and all that can be offered at present is general supportive care for the patient and family with hospitalization in the later stages. The duration of illness in sporadic patients is very short with a mean of 3 to 4 months. However, in some of the inherited cases the duration can be 20 years or more. (30) The prion diseases are now perhaps the best understood of the degenerative brain diseases and the development of rational treatments is appearing realistic. In particular, the development of drugs which selectively bind PrP Sc or which bind to PrPC to inhibit its conversion might be able to block prion propagation. Improved early diagnosis will be crucial.

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