Transmissible spongiform encephalopathies, including CJD and variant CJD (Guidelines)

Objectives

Minimise exposure risk from transmissible spongiform encephalopathies
Ensure appropriate guidance is followed for any potentially or exposed equipment in order to minimise the risk to patient/clients, staff and visitors from Healthcare Associated Infections (HCAIs);
Provide clear guidance and direction through the dissemination of standardised guidance.

Audience

All NHS Highland
Primary and Secondary care
Adults and children

Transmissible spongiform encephalopathies (TSEs), otherwise known as prion diseases, are rare, neurodegenerative diseases affecting the central nervous system (CNS), that occur in humans and certain other mammals.

Although TSEs are not contagious, they are experimentally transmissible by inoculation and in some cases by oral challenge. Transmission of TSEs to humans has occurred from both human and bovine sources, resulting in iatrogenic CJD and variant CJD respectively.

CJD (Creutzfeldt-Jakob disease) is the abbreviation used throughout this document as the other TSEs are very rare and the best practice principles will apply equally to these conditions.

CJD is a progressive, fatal neurological disease that belongs to a wider group of neurodegenerative disorders known as transmissible spongiform encephalopathies (TSEs) or prion diseases. TSEs affect humans and animals. Traditionally, there are three aetiological categories of CJD.

Sporadic CJD (85 to 90% of cases) is of unknown aetiology. Sporadic CJD has a worldwide distribution, with a relatively uniform annual incidence of about 1 in 1 million people.
Inherited CJD (10 to 15% of cases) is associated with coding mutations, insertions or deletions in the prion protein gene.
Iatrogenic CJD (less than 1% of cases) arises from accidental exposure to human prions through surgical or medical procedures.

CJD patients typically present with rapidly progressive dementia, usually accompanied by myoclonus and cerebellar ataxia. Most patients die within 4 months of disease onset, in a mute and immobile state.

A variant form of CJD was identified in 1996, which was described as being identical to Bovine Spongiform Encephalopathy (BSE). It was thought to have been transmitted to humans by eating contaminated meat products. Other instances of transmission of CJD have been described via receiving blood products and occasionally through instruments contaminated with CJD. Unlike micro-organisms, prions are not destroyed by conventional methods of decontamination and sterilisation, therefore a risk assessment has to be undertaken before any clinical procedure where there is considered to be a risk of potential transmission.

Screening of possible CJD prior to surgery or endoscopy

The main implication for NHS Highland regarding TSE, including CJD and vCJD is the screening of patients undergoing surgical procedures and endoscopy in order that the re-usable devices involved in the procedures are managed appropriately.

This screening is carried out by asking all patients about to undergo any elective or emergency surgical or endoscopic procedure:

“Have you ever been notified that you are at increased risk of CJD or vCJD for public health purposes?”

The appropriate action to take to the answer is given in annexe J of the UK TSE guidance:

Management of patients with possible or confirmed CJD

Further information and guidance on all aspects of managing patients together with specific advice for relevant specialities is given within the UK TSE guidance:

Specific advice around infection control management is given within the UK TSE guidance:

Caring for symptomatic persons at home

Those caring for patients at home should be advised of the standard infection prevention and control practices that would apply. Provision should be made with the local authority for the removal and disposal of clinical waste including sharps.

Last offices / Care after death

Guidance on dealing with bodies of patients with or at 'increased risk' of CJD or vCJD can be found in Annexe H of the UK TSE guidance.

Roles and responsibilities

All care providers have a responsibility to:

Apply the principles of this guidance
Ensure their knowledge is updated in relation to the care of a patient with TSE and the management of the environment and medical devices/equipment exposed to TSE.
Report to line managers incidents that may have resulted in cross contamination.

All departmental managers have a responsibility to:

Ensure all staff have access to, and adhere to, this guidance
Ensure if appropriate that all staff have education/ training on the principles of managing patients with TSEs.
Ensure that adequate resources are in place to allow for the recommended infection control measures such as Personal Protective Equipment (PPE) to be implemented.
Support staff in any corrective action or interventions if an incident occurs that may have resulted in cross-contamination

Infection Prevention and Control team must:

Provide support to staff seeking advice when adhering to this guidance;
Act as a resource for guidance and support when advice on TSE / CJD is required.
Provide advice on individual risk assessments for TSE / CJD decisions.
Keep this guidance up to date
Act as a resource on the advice contained within national guidance produced by Public Health Scotland (PHS) and UK Health Security Agency (UKHSA)

Patient's clinician must:

Report any patient suspected on clinical grounds of having vCJD / CJD to the Clinical Team at the National Surveillance Unit in Edinburgh, Tel: 0131 332 2117.

Patients identified as 'at risk' of CJD should be reported to the Public Health Protection Unit (PHPU), Tel: 0141 201 (6)4917 e-mail: PHPU@ggc.scot.nhs.uk, who will report them to ARHAI.

Monitoring document compliance and effectiveness

Managers will be responsible for monitoring the compliance of staff with this guidance. Managers should seek advice from Infection Prevention and Control if they have queries arising from this guidance.

The Infection Prevention and Control team will monitor the effectiveness of this guidance by reviewing the actions undertaken by the clinical teams should any cases be identified.

Precautions for reusable instruments for surgical procedures on patients with or at increased risk of CJD, vCJD and other human prion disease

Abbreviations

BSE: Bovine Spongiform Encephalopath
CJD: Creutzfeldt-Jakob disease
COIC: Control of Infection Committee
CNS: Central nervous system
HCAI: Health Care associated infection
IPC: Infection Prevention and Control
IPCT: Infection Prevention and Control Team
PHS: Public Health Scotland
PPE: Personal Protective Equipment
TSE: Transmissible spongiform encephalopathies
UKHSA: UK Health Security Agency
vCJD: variant Creutzfeldt Jakob Disease

Last reviewed: 30/10/2025

Next review date: 30/10/2028

Author(s): Infection Prevention and Control Department.

Version: 1

Approved By: TAM subgroup of the ADTC

Reviewer name(s): C Stokoe, Infection Control Manager.

Document Id: TAM715

References

Advisory Committee on Dangerous pathogens. Spongiform Encephalopathy Advisory Committee (2003) Transmissible spongiform Encephalopathy Agents: safe working and the prevention of infection Guidance for Health care workers. HMSO

Medical Devices Agency Decontamination of Endoscopes July 2002 MDA DB (2002)05

National Institute for Health and Clinical Excellence: patient safety and reduction of risk of transmission of Creutzfeldt-Jakob disease (CJD via interventional procedures. November 2006 (Guidance endorsed by NHS QIS for implementation by NHS Scotland)

“Endoscopy and individuals at risk of vCJD for public health purposes” A consensus statement from the British Society of Gastroenterology Decontamination Working Group and the ACDP TSE Working Group Endoscopy and vCJD Sub-Group (November 2005)

THE NATIONAL CJD RESEARCH & SURVEILLANCE UNIT (NCJDRSU) | National CJD Research & Surveillance Unit

National Infection Prevention and Control Manual: A-Z Pathogens