Transmissible Spongiform encephalopathy
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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.
- It is characterized by
- Progressive cerebellar ataxia (Dysarthria, Incoordination)
- Loss of control over muscle movements
Sign / Symptoms
- Dementia: Memory changes, Confusion
- Personality changes
- Problems with movement: Myoclonus
- Depression
- Ataxia: Lack of coordination, Unsteady gait
- Unusual sensations,
- Insomnia,
Features
- Spongiform change
- Neuronal loss
- Astrocytosis
- Amyloid plaque formation