Please note: This is a basic revision aid, not a comprehensive and reliable statement of medical fact. These notes should not be used to guide treatment. For reliable information and advice, consult a qualified practitioner.
Similar prion diseases include Gerstmann-Straussler-Scheinker syndrome (GSS) and Kuru.
1:1000000. Very rare despite media prominence. Prevalence may change depending on spread of nvCJD. There were 26 confirmed cases of nvCJD in the UK in 2000.
Late middle age for classic CJD, while nvCJD tends to appear in young adults.
The molecular cause is a prion protein (PrP). 10-15% of classic CJD cases seem to be caused by familial transmission (autosomal dominant) of a defective PrP gene. The rest are thought to be due to a spontaneous mutation of the PrP gene. The prion protein is highly resistant to heat and chemical neutralisation. Can be transmitted by dura mater grafts, contaminated surgical instruments and growth hormone derived from infected human pituitary glands. Apparently, no transmission from mother to child in pregnancy or breast feeding. nvCJD is thought to be transmitted through consumption of infected foodstuffs -- central nervous system (CNS) matter from Bovine Spongiform Encephalopathy (BSE) infected cattle. There is some concern about cross-species transmission to sheep and a suggestion that if sheep were to become infected, the prion would not be restricted to the CNS, but could be carried in muscle tissue and elsewhere.
Spongiform encephalopathy -- cortical degeneration.
Initial symptoms may be psychiatric, with depression as a common feature. Blurred vision, dizziness, fatigue and insomnia are also common early symptoms. Cognitive impairment, cerebellar syndrome with gait and limb ataxia, limb weakness and dysphasia typically follow, with further degeneration involving weight loss, incontinence and severe dementia.
Approx. 6 months for classic CJD, 23 months for nvCJD
Symptomatic treatments only at present. Genetic counselling in the case of classic CJD, due to the small risk of familial transmission. the CJD Support Network can supply a Case Coordinator on request.
Likely problem areas:
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Last updated: 17th March 2004
Author: Mike Griffin PGDipOT, SROT
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