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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).
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).
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:
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Substantiated information by:
Jordi Sarto Alonso
Raquel Sanchez del Valle DíazNeurology Head of Department
Published: 21 December 2021
Updated: 21 December 2021
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