CreutzfeldtJakob Disease Atif Chohan Alex Brown What is
Creutzfeldt-Jakob Disease Atif Chohan & Alex Brown
What is CJD? • • Degenerative brain disorder Uncommon, usually fatal Appears in older people, short duration First described in 1920 – Hans Gerhard Creutzfeld/Alfons Maria Jakob
3 Categories of CJD • Sporadic CJD (85%) – Individual has no known risk factors • Hereditary CJD (10 -15%) – Family history – positive for genetic mutation • Acquired CJD (<1%) – Exposure to brain/nervous system tissue – Usually medical procedures (iatrogenic)
Signs & Symptoms • Dementia • Muscular coordination, personality changes, vision • Myoclonus, blindness • Enter coma, often contract infection • Early stage similar to Alzheimer’s/Huntington’s • “Human form of Mad Cow disease”
Causes of CJD • Prions – Proteins, 2 forms – normal/infectious – Same amino acid sequence, folded differently – Infectious prions aggregate – Can be transmitted to offspring • Unusual ‘slow virus’ – No virus ever isolated
Prions • Prions invade brain
• Nerve cell death (causes symptoms) • Tiny holes in tissue – ‘spongiform’
Transmission of CJD • Contaminated harvested human brain products • Inherited • Human Growth Hormone • Cannibalism • Blood, plasma? • Not transmitted by air/contact
Epidemiology • • Very rare – 1 in a million per year Mostly 55 -75 years (except variant CJD) 90% fatality rate within 1 year In United Kingdom: – 1860 confirmed deaths since 1990 (1454 sporadic) – 68 deaths in 2013 so far (4 th Nov) – Zero confirmed cases still alive
References Ø http: //www. cjd. ed. ac. uk/documents/figs. pdf Ø http: //www. rightdiagnosis. com/c/creutzfeldt_ jakob_disease/stats. htm Ø http: //en. wikipedia. org/wiki/Creutzfeldt%E 2% 80%93 Jakob_disease Ø http: //www. ninds. nih. gov/disorders/cjd/detai l_cjd. htm
- Slides: 10