The National Prion Clinic team at UCL have developed a rating scale for prion diseases, that will have major implications in future treatment trials. In a paper published in the scientific journal Brain (click here to read), the team described a precise way to track how patients with prion disease are progressing.
The use of the MRC Prion Disease Rating Scale or “MRC Scale” in short form, in the National Monitoring Prion Cohort has revealed there are three distinct patterns of change:
  1. slow progression associated with genetic forms of disease
  2. rapid progression associated with any form of the disease
  3. a proportion of patients that survive in a comatose state sometimes for prolonged periods of time. 
The paper is based on follow-on work from a MRC funded clinical trial PRION-1 (2004-2007), using the anti-malarial drug – quinacrine, which concluded that there was a need for a definitive prion disease rating scale. The PRION-1 study raised lots of questions, in particular about how to measure the progress of patients through the disease. Some rating scales we have used in the past were poor at measuring patient progression, though questions about every-day activities seemed to match clinical reality.
The new rating scale is based on interviews with 71 families, we’ve taken into account the experience of the disease from the perspective of patients, their relatives and carers to find out what are the important symptoms of the disease. Questions focus on speech, memory, continence, mobility and self-care, which are all important aspects of how the disease impacts on families. We appreciate and acknowledge the contribution of carers and relatives to this research work.