Dementia Creutzfeldt-Jakob disease-related

The prion diseases are a closely related group of neuro-degenerative conditions which affect both humans and animals. They have previously been described as the subacute spongiform encephalopathies, slow virus diseases and transmissible dementias, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and the human prion diseases, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and kuru. Prion diseases are... 

A misfolded protein appears to be the causative agent of a number of rare degenerative brain diseases in mammals. Perhaps the best known of these is mad cow disease (bovine spongiform encephalopathy, BSE), an outbreak of which made international headlines in the spring of 1996. Related diseases include kuru and Creutzfeldt-Jakob disease in humans, scrapie in sheep, and chronic wasting disease in deer and elk. These diseases are also referred to as spongiform encephalopathies, because the diseased brain frequently becomes riddled with holes (Fig. 1). Typical symptoms include dementia and loss of coordination. The diseases are fatal. 

Steroid-responsive encephalopathies can be considered vasculitic or non-vasculitic. Clinical features are suggestive of Creutzfeldt-Jakob disease (CJD), dementia with Lewy bodies (DLB), and parkinsonism, but pathological examination revealed only AD-related findings without evidence of Lewy bodies or prion disease in most cases. AD is not diagnosed in life due to the atypical clinical features, lack of hippocampal atrophy on brain imaging, and a dramatic symptomatic response to steroids [97], Some cases of new-variant CJD may also be misdiagnosed as AD. 

Consider rarer dementias if unusual (particularly neurological) symptoms frontotemporal, dementia in Parkinson s disease, Huntingdon s disease (family histoiy, chorea), Creutzfeldt Jakob Disease, AIDs-related encephalopathy, neurosyphilis, progressive supranuclear palsy. 

---