Transmissible spongiform encephalopathy overview

Jump to: navigation, search

Transmissible spongiform encephalopathy Microchapters


Patient Information


Historical Perspective




Differentiating Transmissible spongiform encephalopathy from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Neurocognitive Disorder Due to Prion Disease


History and Symptoms

Physical Examination

Laboratory Findings



Other Imaging Findings

Other Diagnostic Studies


Medical Therapy

Primary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Transmissible spongiform encephalopathy overview On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides


American Roentgen Ray Society Images of Transmissible spongiform encephalopathy overview

All Images
Echo & Ultrasound
CT Images

Ongoing Trials at Clinical

US National Guidelines Clearinghouse

NICE Guidance

FDA on Transmissible spongiform encephalopathy overview

CDC on Transmissible spongiform encephalopathy overview

Transmissible spongiform encephalopathy overview in the news

Blogs on Transmissible spongiform encephalopathy overview

Directions to Hospitals Treating Transmissible spongiform encephalopathy

Risk calculators and risk factors for Transmissible spongiform encephalopathy overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Please help WikiDoc by adding more content here. It's easy! Click here to learn about editing.


Transmissible spongiform encephalopathies are a group of progressive conditions that affect the brain and nervous system of humans and animals and are transmitted by prions. Mental and physical abilities deteriorate and myriad tiny holes appear in the cortex causing it to appear like a sponge (hence 'spongiform') when brain tissue obtained at autopsy is examined under a microscope. The disorders cause impairment of brain function, including memory changes, personality changes and problems with movement that worsen over time. Prion diseases of humans include classic Creutzfeldt-Jakob disease, new variant Creutzfeldt-Jakob disease (a human disorder related to mad cow disease), Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia and kuru. These conditions form a spectrum of diseases with overlapping signs and symptoms.


Unlike other kinds of infectious disease which are spread by microbes, the infectious agent in TSEs is a specific protein called prion protein. Misshaped prion proteins carry the disease between individuals and cause deterioration of the brain. TSEs are unique diseases in that their aetiology may be genetic, sporadic or infectious via ingestion of infected foodstuffs and via iatrogenic means (e.g. blood transfusion) (reviewed in Prusiner, 1991; Collinge, 2001).

Epidemiology and Demographics

These spontaneous disorders in humans are very rare affecting only about one person per million worldwide each year.