Diseases of the central nervous system caused by prions
Diseases of the central nervous system (CNS) caused by prions are progressive, fatal conditions that affect brain function. Prions are misfolded proteins that can transmit their misfolded shape onto normal variants of the same protein. Here’s an overview of CNS diseases caused by prions.
Prion diseases, or transmissible spongiform encephalopathies, are a group of life-threatening neurodegenerative diseases characterized by progressive dementia and motor dysfunction. When infected, behavior changes, personality traits alter, seizures occur, vision and hearing impairments arise, and movement and coordination problems develop. Typically, survival is less than a year from the onset of the first symptoms.
Common Prion Diseases
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Creutzfeldt-Jakob Disease (CJD):
- Symptoms: Rapid onset of dementia, memory loss, personality changes, hallucinations, and motor dysfunction.
- Types: Sporadic (sCJD), familial (fCJD), iatrogenic (iCJD), and variant (vCJD, linked to Bovine Spongiform Encephalopathy – BSE or mad cow disease).
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Gerstmann–Sträussler–Scheinker Syndrome (GSS):
- Symptoms: Ataxia (loss of coordination), dementia, and other neurological symptoms.
- Inheritance: Inherited as an autosomal dominant trait.
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Fatal Familial Insomnia (FFI):
- Symptoms: Severe insomnia, hallucinations, delirium, and eventually, dementia.
- Inheritance: Autosomal dominant trait.
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Kuru:
- Symptoms: Tremors, loss of coordination, difficulty walking, and emotional instability.
- Transmission: Historically transmitted through cannibalistic rituals in Papua New Guinea.
Source | Author Doctor Nikas Samuolis, reviewed by Prof. Virginijus Šapoka | Vilnius University | Faculty of Medicine | Head of the Department of Internal Medicine, Family Medicine, and Oncology