Prion diseases are uniformly fatal, often rapidly progressive, neurodegenerative diseases resulting from the transformation and accumulation of a native protein, the prion protein, into an abnormally shaped protein, called the Prion. The history of Prion disease commenced in the year 1920, when Hans Gerhard Creutzfeldt reported a 22 year old woman with a mysterious and progressive focal syndrome of CNS that was clinically identified by psychomotor abnormalities and cortical symptoms. Researchers have shown that the disease occur in most of the developed world at a rate of 1 to 1.5 cases per million per year. The treatment is aimed at alleviating the symptoms and making the patient as comfortable as possible. The preferable recommendations in prion disease is Genetic Counselling involving pre and post test counselling of patients by including an assessment of capacity and discussion of the risks and benefits of testing
