Creutzfeldt-Jakob disease (CJD)

Background

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Creutzfeldt-Jakob disease (CJD) is a rare and fatal degenerative brain disease. It's one of a group of diseases called Transmissible Spongiform Encephalopathies (TSEs) that affect humans and animals. TSEs are thought to be caused by the build up in the brain of an abnormal form of the naturally occurring prion protein. Unfortunately, there's currently no treatment or cure for CJD.

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When CJD occurs, local NHS boards and local health protection teams are responsible for its:

  • investigation
  • assessment
  • management
  • patient notification

Further information on the different types of CJD can be found on the NHS website.

Guidance

National guidance on CJD Incident management has been developed to support this management process and can be found on the Public Health England website.

The guidance provides advice and information for the public health follow up that's required and should be used for:

  • a newly diagnosed or suspected case of CJD
  • a person at increased risk of CJD
  • a surgical procedure carried out on a patient with CJD
  • a surgical procedure carried out on a patient at increased risk of CJD where TSE infection control guidelines weren't followed

A CJD incident management guidance document and forms has also been produced by Public Health England and it's for use by:

  • trusts
  • hospitals
  • local health protection teams

For all infection prevention and control guidance visit the A-Z ​pathogens section of the National Infection and Prevention Control Manual.

Publications

Public Health England has produced CJD leaflets for patients identified as at increased risk of the disease and for healthcare professionals involved in their care or notification.

Data and surveillance

In 1996, a new form of CJD known as variant CJD (vCJD) was first identified and it's strongly linked to exposure, probably through food, to a TSE of cattle called Bovine Spongiform Encephalopathy (BSE).

To help prevent the spread of CJD in healthcare, a series of public health measures have been implemented which include:

  • improving the standards and processes for decontaminating instruments
  • taking special infection control measures in relation to instruments when operating on patients with CJD or at increased risk of CJD
  • measures to protect the blood supply
  • excluding transfused donors from the living bone donation programme

We collect information on the number of people in Scotland who've been identified as being at increased risk of contracting CJD. This information is collected by sending GPs a letter and form and helps to determine how the number of people who've contracted vCJD develops over time.