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CREUTZFELDT-JAKOB DISEASE: EIGHT CASES IN MID-ESSEX FROM 2010 TO 2015, A REFLECTION OF A HIGHER INCIDENCE THAN CURRENTLY REPORTED? | 55750
Epidemiology: Open Access
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Creutzfeld-Jakob disease (CJD) is a fatal neurodegenerative disorder caused by the accumulation of prion protein in neural
tissue. The most common of these is sporadic CJD (sCJD), with an estimated incidence of 0.4-1.8/million/year. This case
series looked at a high incidence of sCJD in the catchment area of Broomfield Hospital, Essex, UK, where 8 patients were
diagnosed between 2010 and 2015. This incidence of 3.72/million/year is significantly higher than the UK national average of
1.07/million/year from 1990 to 2014 (p=0.011).
The aim of this presentation is to look at the potential causes of this high incidence, which include an iatrogenic cause
with a common origin, high diagnostic vigilance, a disease incidence higher than previously considered, or chance occurrence.
None of the patients had surgery, making iatrogenic cause unlikely. CJD affects predominantly the elderly population and
shows clinical similarity to other neurological conditions, potentially leading to misdiagnosis. Initial changes on MRI were not
found on 7 of the 8 patients, though retrospective analysis showed changes visible in 6 of those scans, exhibiting the difficulties
in diagnosis. The incidence of sCJD in Britain has been increasing, which is a trend observed in other European countries,
considered to be due to increased accuracy in diagnosis and surveillance. We propose that the raised incidence of sCJD in
Mid-Essex could reflect a higher true incidence of CJD than is currently reported, as a result of underdiagnosis of the disease.
Simon Cavinato has been Graduated from Barts and The London School of Medicine and Dentistry as Batchelor of Medicine and Surgery, and Batchelor of Science in Biomedical Science from the University of Leeds. He then complete foundation training in Princess Alexandra Hospital, Harlow and Queens Hospital, Romford. Presently he has been working at St George’s Hospital in London as Clinical Fellow in Intensive Care.