A third patient with a rare PRNP mutation, G131V, was also studied for transmission . In this patient, the disease onset was at age 36 and the patient died after 15 years of progressive clinical disease including personality changes, memory impairment, loss of cognition, tremor, ataxia, myoclonus, and bradykinesia. Neuropathology showed numerous deposits of PrP amyloid in gray matter of cerebrum, cerebellum and midbrain without evidence for spongiform degeneration. Another patient with the same mutation was reported previously .