Catalog

Familial Alzheimer-like prion disease

No MMS code

Link to WHO Definition

Familial Alzheimer-like prion disease – http://id.who.int/icd/entity/1297025427

Familial Alzheimer-like prion disease is an exceedingly rare form of prion disease characterized by the neuropathological features of Alzheimer disease including memory impairment and depression, related to abnormal prion protein (PrP) caused by a pathogenic variant in the PRNP gene. Patients present with a prolonged, atypical course (absence of myoclonus or ataxia) unlike other forms of prion disease with severe neurofibrillary tangle pathology and high levels of cerebral amyloidosis.