G_IPC_42 Transmissible Spongiform Encephalopathy Agents

The purpose of this guidance is to provide healthcare workers who provide clinical care and/or practice in primary care and community settings with a practical working document to underpin safe working practices in relation to Transmissible Spongiform Encephalopathy (TSE).

Transmissible spongiform encephalopathies (TSEs), sometimes known as prion diseases are fatal degenerative brain diseases which occur in humans and certain animal species.  A common feature of all TSEs is the appearance of microscopic vacuoles (holes) in the grey matter of the brain, resulting in a sponge-like appearance.

Some TSE's have a protracted pre-clinical phase.  This is followed by rapidly progressive dementia, loss of memory and intellect, personality changes or progressive unsteadiness and clumsiness.

TSE in humans is relatively rare. Known human TSE's include:

  • Creutzfeldt-Jakob disease (CJD) including classical, sporadic, familial, iatrogenic and new variant
  • Gerstmann-Straussler-Sheinker syndrome (GSS)
  • Fatal familial insomnia (FFI)
  • Kuru

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