Gerstmann-Straussler-Scheinker Syndrome
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Gerstmann-Straussler-Scheinker Syndrome is a transmissible spongiform encephalopathy in humans. It is genetic, and the mutations that cause it include P102L and A117V. Symptoms usually develop between 35 and 55. Once symptoms start, people generally live 5 years, but it can range from 1.5-12 years. Symptoms include dementia, poor reflexes, ataxia, issues with speech, and cognitive disfunction.
References
https://rarediseases.info.nih.gov/diseases/7690/gerstmann-straussler-scheinker-disease