title: Human prion diseases.
abstract: Prion diseases is another name for a group of 'transmissible spongiform encephalopathies'. Creutzfeldt-Jakob disease, the first prion disease described in humans, occurs in sporadic, familial or iatrogenic form. Other transmissible spongiform encephalopathies in humans such as familial Creutzfeldt-]akob disease, Gerstmann-Sträussler-Scheinker disease and fatal familial Insomnia have been shown to be associated with specific prion protein gene mutations. In 1996, a new variant of Creutzfeldt-Jakob disease was reported in the United Kingdom among young patients with unusual clinical features and unique neuropathological findings. This new form could be due to transmission to humans of the agent causing bovine spongiform encephalopathy. While examination of brain tissue is the key to making a diagnosis, it is not always possible antemortem. Immunological tests such as ELISA or western blot assays along with tests for 1 4-3-3 protein in the cerebrospinal fluid remain the main tools of diagnosis. Conventional disinfection and sterilization practices are Ineffective for these agents. The unusual properties of prions pose a challenge for treatment, surveillance and control of these diseases.
authors.affiliation: Department of Microbiology, Institute of Human Behaviour and Allied Sciences, PO Box 9520, Jhimill, Dilshad Garden, Delhi 110095, India. drrajeevthakur@hotmail.com
authors.forename: Rajeev
authors.initials: R
authors.lastname: Thakur
authors.forename: Yasmeen Marbaniang
authors.initials: YM
authors.lastname: Vincent
authors.forename: Sujata
authors.initials: S
authors.lastname: Chaturvedi
mesh_terms: D000818:Animals; D006801:Humans; D011159:Population Surveillance; D017096:Prion Diseases / Q000453:epidemiology / Q000235:genetics / Q000473:pathology* / Q000517:prevention & control