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Thomas Li

Prion disease

Types

  • Sporadic (85-95%)

  • Familial (5-15%)

  • Iatrogenic (<5%)

    • use of cadaveric human growth hormone, dural  graft transplant, corneal transplant, liver transplant and contaminated neurosurgical instrument

  •  Variant

    • > 155 cases reported worldwide since 1995

    • most in UK

    • likely bovine-to-human transmission of bovine spongiform encephalopathy

    • 2 possible cases of transfusion related vCJD

Epidemiology

  • Around 1 case per 1,000,000 population per year

  • Mean age of onset 57 – 62 years

  • Younger age of onset in variant or iatrogenic CJD

Clinical features

  • Rapidly progressive mental deterioration

  • Dementia, behavioural changes, higher cortical function like memory, judgement difficulties, mood changes

  • Myoclonus (present in > 90% of cases)

  • Extrapyramidal signs (eg hypokinesia) or cerebellar manifestations (60%)

  • Signs of corticospinal tract involvement (40-80%)

  • Cranial nerve abnormalities, sensory abnormalities or peripheral nervous system involvement are not prominent features

 vCJD

  • Younger age at onset (29 vs 65 years)

  • Less rapid progression with long duration of illness (14 months vs 4.5 months)

  • Initial psychiatric and sensory symptoms

  • All were potentially exposed to contaminated bovine animals during 1980s

Imaging

  • CT scan – cortical atrophy , ventricular dilatation, to exclude other lesion

  • MRI – 80% showed increased density at basal ganglia on T2 or proton weighted images

WHO diagnostic criteria

http://www.who.int/emc-documents/tse/whocdscsraph2003c.html

Infection control

  • Not transmitted by direct contact, droplet or airborne

  • Iatrogenic CJD due to exposure to infectious brain, dura mater, pituitary, eye

Usual social and clinical contact

  • Unlikely to be risky, no need for isolation room

Body fluids

  • saliva, body secretions or excreta – no infectivity demonstrated so far

  • standard precaution against body fluid

Blood, biopsy and lumbar puncture

  • Potentially infective

  • Disposable gloves, aprons, eye protection when splashing may occur

  • Disposable instruments

Spillage

  • Dilute with water then clean with high concentration sodium hypochlorite

Potential exposure

  • Sharps injury

    • Wound gently encouraged to bleed , gentle washing with warm soapy water, rinsed and dried and covered with water proof dressing, avoid scrubbing

  • Eye or mouth contamination – thorough irrigation

  • Report to accident supervisor

UK guidelines

http://www.advisorybodies.doh.gov.uk/acdp/tseguidance/index.htm

Further reading

http://www.cjd.ed.ac.uk/

http://www.cdc.gov/ncidod/diseases/cjd/cjd_inf_ctrl_qa.htm

http://gut.bmjjournals.com/cgi/content/full/50/6/888

© Thomas Li, March 2005


©Charles Gomersall, April, 2014 unless otherwise stated. The author, editor and The Chinese University of Hong Kong take no responsibility for any adverse event resulting from the use of this webpage.
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