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Human prion diseases have different names according to the signs and symptoms presented by the patient:  

Creutzfeldt-Jakob disease is the most common, and accounts for 90% of cases. Symptoms begin within days or weeks in previously healthy middle aged people. Death usually occurs around 6 months after the onset of symptoms.  The most frequent initial symptoms (in order of progression) are:  

  • Cognitive problems; affecting memory, perception and problem solving.
  • Movement coordination and gait disorders.  
  • Motor disorders or stiffness.
  • Involuntary muscle tremors or twitching (myoclonus).
  • Visual disturbances.  
  • The patient loses their connection with their environment, and cannot speak or move.  

Fatal insomnia (familial or sporadic): most cases are due to a genetic mutation. Symptoms usually start around the age of 40-50. Progression is rapid, and patients die within 18 months of the onset of symptoms.  

  • Disturbance of biological cycles.  
  • Sleep disturbances ranging from daytime tiredness to non-restful sleep at night, interrupted by movements or sleep talking (somniloquy).
  • Cognitive problems.
  • Difficulties walking.
  • Sweating disturbances, palpitations, fever, changes in blood pressure (dysautonomia).  
  • Brief, rapid involuntary movements (myoclonus).
  • Changes in the voice.  

Gerstmann-Sträussler-Scheinker disease or octapeptide insertion disease. This is a less common prion disease.  At the beginning a slower course predominates:

  • Gait disturbance.
  • Cognitive dysfunction.

Substantiated information by:

Jordi Sarto Alonso
Raquel Sanchez del Valle Díaz

Published: 21 December 2021
Updated: 21 December 2021

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