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<p>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.</p><p> </p><p>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:</p><p>
</p><p><a href="http://www.cjd.ed.ac.uk/surveillance/referral-system" target="_blank">http://www.cjd.ed.ac.uk/surveillance/referral-system</a></p><p>
</p><p>The Department and the Scottish Government have funded the development of diagnostic
tests that combined with improved interpretation of neuroimaging has improved diagnostic
accuracy.</p><p> </p><p>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.</p><p> </p><p>Number of sCJD 2014-18 in the UK by year of death (as
at January 2019).</p><table><tbody><tr><td><p>Year of death</p></td><td><p>Number</p></td></tr><tr><td><p>2014</p></td><td><p>99</p></td></tr><tr><td><p>2015</p></td><td><p>105</p></td></tr><tr><td><p>2016</p></td><td><p>119</p></td></tr><tr><td><p>2017</p></td><td><p>120</p></td></tr><tr><td><p>2018</p></td><td><p>125</p></td></tr></tbody></table><p>Source:
NCJDRSU: <a href="https://www.cjd.ed.ac.uk/sites/default/files/figs.pdf" target="_blank">https://www.cjd.ed.ac.uk/sites/default/files/figs.pdf
</a></p><p> </p><p>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:</p><p> </p><p><a href="http://www.cjd.ed.ac.uk/sites/default/files/report26.pdf"
target="_blank">http://www.cjd.ed.ac.uk/sites/default/files/report26.pdf</a></p><p>
</p><p>The NCJDRSU website also provides information on the different types of CJD
at the following link:</p><p> </p><p><a href="http://www.cjd.ed.ac.uk/surveillance/diagnosis-and-testing"
target="_blank">http://www.cjd.ed.ac.uk/surveillance/diagnosis-and-testing</a></p><p>
</p><p>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.</p>
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