CJD

(asked on 8th January 2019) - View Source

Question to the Department of Health and Social Care:

To ask the Secretary of State for Health and Social Care, what steps his Department is taking to ensure that the symptoms of sporadic Creutzfeldt-Jakob disease are identified at the earliest opportunity.


Answered by
Steve Brine Portrait
Steve Brine
This question was answered on 16th January 2019

Early diagnosis of sporadic Creutzfeldt-Jakob Disease (sCJD) is challenging as the disorder is rare, and symptoms are non-specific, mimicking those seen in more common neurodegenerative, neuroinflammatory, and infectious disorders. Rapidity of progression and lack of response to treatment often leads clinicians to suspect a CJD diagnosis.

The Department and the Scottish Government fund the National CJD Research and Surveillance Unit (NCJDRSU) to provide inter-disciplinary clinical and laboratory expertise (neurology, neuroradiology, neuropathology, biochemistry, and epidemiology) enabling early and accurate diagnosis of CJD. Clinicians from across the United Kingdom can discuss cases at any time. Highly trained specialists assess suspected cases locally within five days of referral and conduct structured assessments (clinical history, examination, and review of investigation results) before establishing diagnoses based on validated criteria. A consultant neuroradiologist at the NCJDRSU assesses neuroimaging for patients throughout the UK with a suspicion of CJD. The unit also provides a national diagnostic cerebrospinal fluid (CSF) 14-3-3/RT-QuIC service and will arrange collection of CSF samples with prompt results. Information on how clinicians can refer cases to the NCJDRSU is available at the following link:

http://www.cjd.ed.ac.uk/surveillance/referral-system

The Department and the Scottish Government have funded the development of diagnostic tests that combined with improved interpretation of neuroimaging has improved diagnostic accuracy.

The NCJDRSU collects data on the number of people diagnosed with sCJD in the UK; break downs by of data for smaller geographies are not published. The following table shows a breakdown of the number of individuals, by year of death between 2014 and 2017. Date of diagnosis are considered synonymous for the purpose of reporting.

Number of sCJD 2014-18 in the UK by year of death (as at January 2019).

Year of death

Number

2014

99

2015

105

2016

119

2017

120

2018

125

Source: NCJDRSU: https://www.cjd.ed.ac.uk/sites/default/files/figs.pdf

The NCJDRSU presents data regularly at clinical meetings and publishes in peer reviewed medical journals accessed by medical professionals, as well as producing a detailed annual report at the following link:

http://www.cjd.ed.ac.uk/sites/default/files/report26.pdf

The NCJDRSU website also provides information on the different types of CJD at the following link:

http://www.cjd.ed.ac.uk/surveillance/diagnosis-and-testing

sCJD is a rapidly progressive neurological disorder with an average time from first symptom onset to death of four months. The majority (88%) of individuals with sCJD over the past five years were assessed by clinicians from the NCJDRSU in life. After formal referral to NCJDRSU the average time to confirmation of the diagnosis was three days.

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