Prion diseases

Prion diseases are caused by the accumulation of dysmorphic proteins named prions, and are characterized by progressive neurological decline and eventual death. In humans, these diseases include:

They are all mediated by prions: abnormally folded self-catalyzing endogenous proteins which accumulate within the nervous system. Diagnosis has been problematic as imaging findings are variable and CSF protein analysis (14-3-3 protein) insensitive.

Brain biopsy is accurate but is fraught with difficulties as equipment needs to be discarded (prions have been found to survive standard autoclaving procedures) and staff precautions are difficult to implement.

DWI changes are the earliest imaging manifestation prior to FLAIR and cerebral volume changes. Typically, cortical, and deep grey matter (caudate, thalamus and putamen) demonstrates increased signal, not confined to vascular territories.

There is no curative treatment available for any prion disease.

Neurodegenerative diseases

Neurodegenerative diseases are legion and their classification just as protean. A useful approach is to divide them according to underlying pathological process, although even using this schema, there is much overlap and thus resulting confusion.

Infections of the central nervous system

Article information

rID: 4043
Synonyms or Alternate Spellings:
  • Prion encephalopathies
  • Prion encephalopathy

ADVERTISEMENT: Supporters see fewer/no ads

Cases and figures

  • Case 1: sporadic CJD
    Drag here to reorder.
  • Case 1: sporadic CJD
    Drag here to reorder.
  • Updating… Please wait.

     Unable to process the form. Check for errors and try again.

     Thank you for updating your details.