Prion diseases

Human prion diseases, characterised by progressive neurological decline, and eventual death include:

Pathology

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

Radiographic features

MRI

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.


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.

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Article Information

rID: 4043
Section: Pathology
Synonyms or Alternate Spellings:
  • Prion encephalopathy

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Cases and Figures

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