Transmissible Spongiform encephalopathy

Clinic

• TSEs are a group of progressive and fatal conditions that are associated with prions (CJD, NVCJD, GSS, FFI and KRU)
• Its name comes from many tiny holes appearing in the cortex in autopsy.
• Sign / Symptoms are
  • Dementia: Memory changes, Confusion
  • Personality changes
  • Problems with movement: Myoclonus
  • Depression
  • Ataxia: Lack of coordination, Unsteady gait
  • Unusual sensations,
  • Insomnia,

Features

1. Spongiform change
2. Neuronal loss
3. Astrocytosis
4. Amyloid plaque formation