Clinical and neuroimaging characteristics of 14 patients with prionopathy: a descriptive study

Prionopathy is the cause of 62% of the rapidly progressive dementias (RPD) in which a definitive diagnosis is reached. The variability of symptoms and signs exhibited by the patients, as well as its different presentation, sometimes makes an early diagnosis difficult. Patients with diagnosis of definite or probable prionopathy during the period 1999–2012 at our hospital were retrospectively reviewed. The clinical features and the results of the...