Transmissible Spongiform Encephalopathies Affecting Humans

2012/08/14 by Ghanshyam Dudhatra, Avinash Kumar, Chirag M. Modi +3 · 1 citation
Biochemistry, Genetics and Molecular Biology · Neuroscience · Medicine · #Prion Diseases and Protein Misfolding #Neurological diseases and metabolism #Folate and B Vitamins Research

paper · pdf · doi:10.5402/2013/387925

openalex publication_date 2012/08/14 · openalex created_date 2025/10/10 · openalex updated_date 2026/05/21

Abstract

Transmissible spongiform encephalopathies (TSEs) or prion diseases are group of rare and rapidly progressive fatal neurologic diseases. The agents responsible for human prion diseases are abnormal proteins or prion that can trigger chain reactions causing normal proteins in the brain to change to the abnormal protein. These abnormal proteins are resistant to enzymatic breakdown, and they accumulate in the brain, leading to damage. TSEs have long incubation periods followed by chronic neurological disease and fatal outcomes, have similar pathology limited to the CNS including convulsions, dementia, ataxia, and behavioral or personality changes, and are experimentally transmissible to some other species.

Citations

• Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy
• Gerstmann–Sträussler–Scheinker syndrome,fatal familial insomnia, and kuru: a review ofthese less common human transmissiblespongiform encephalopathies
• Kuru in the 21st century—an acquired human prion disease with very long incubation periods
• CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease
• Creutzfeldt‐Jakob disease: Clinical analysis of a consecutive series of 230 neuropathologically verified cases
• Balancing Selection at the Prion Protein Gene Consistent with Prehistoric Kurulike Epidemics

Cited by

• Creutzfeldt–Jakob disease [wikipedia]