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Variably Protease-Sensitive Prionopathy

by Pierluigi Gambetti, MD

Variably protease-sensitive prionopathy is a new form of prion disease, identified in 2008. It appears to be very rare, but how many people are affected is unknown. It typically develops around age 70, and life expectancy is about 24 months.

At first, people usually have changes in mood and behavior. They may lose their inhibitions, have intense feelings of well-being (euphoria), lose interest in their usual activities, or become listless. They may have problems speaking and may become uncoordinated. Mental function is impaired. Walking eventually becomes difficult.