Occupational Therapy Revision Notes:
Creutzfeldt-Jakob Disease (CJD)
and new variant Creutzfeldt-Jakob Disease (nvCJD)

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.

Prevalence

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.

Age at onset

Late middle age for classic CJD, while nvCJD tends to appear in young adults.

Cause

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.

Pathology

Spongiform encephalopathy -- cortical degeneration.

Presentation and symptoms

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.

Prognosis

Approx. 6 months for classic CJD, 23 months for nvCJD

Treatment

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.

Implications for Occupational Therapy

Likely problem areas:

• Challenging behaviour and sometimes aggression
• Increasing mobility problems
• Incontinence
• Difficulty swallowing and drooling
• Inability to communicate
• Inability to recognise people
• Cortical blindness
• Coma
• Practical and emotional support for the affected individual and family members.
• A named keyworker (familiar with local health and social care resources) must be identified at an early stage to coordinate multidisciplinary input.
• High quality information is vital for both the person with CJD and their family.
• Confidentiality is more important than usual due to the high level of media interest in nvCJD.
• Fear of strangers is commonly seen and requires continuity of care to ensure familiar faces.
• Full multidisciplinary input will be required and the OT may be involved in facilitating this by referring on to other services.
• Equipment such as handrails, ramps, bathing equipment, wheelchairs, hoists and special beds are likely to be needed, and must be delivered in a timely manner due to the speed of progression of the disease.
• "National CJD Surveillance Unit (CJDSU) has employed a national care co-ordinator to provide specialist expertise in CJD and act as an information resource for carers and professionals. The co-ordinator is Gordon McLean and he can be contacted at the CJDSU on 0131 537 2129". [source: DoH guidance]
• "From April 1999 to March 2002 the Department of Health has funded a national CJD case co-ordination project run by the CJD Support Network. Mrs Gillian Turner, the case co-ordinator, will attend the first case conference of a patient with CJD by invitation and assist all those present by providing information on effective forms of support, encouraging co-ordination and partnership between services and applying a unique understanding of carers needs. She can be contacted on 01630 673 993." [source: DoH guidance]

Links

• CJD Surveillance Unit (UK)
• Patients with new variant Creutzfeldt-Jakob disease and their families: care and information needs (comprehensive report from CJD Surveillance Unit, UK)
• Diagnosis, Treatment and Care of vCJD Patients Volume 8, Chapter 6 of the report of the BSE Inquiry (also available in PDF format)
• CJD Support Network (UK, part of the Alzheimer's Society)
• Dept. of Health guidance on CJD for healthcare workers (UK)
• Human BSE Foundation (UK, set up by and for families of people with nvCJD)
• CJD Foundation, Inc. (US)
• Medline Plus Medical Encyclopaedia: Creutzfetdt-Jakob Disease

Last updated: 17th March 2004

Author: Mike Griffin PGDipOT, SROT

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