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Abstract Background Variant Creutzfeldt - Jakob disease (vCJD) arose from dietary contamination with bovine-spongiform-encephalopathy (BSE). Because of concerns that vCJD-cases might be missed in the elderly, a feasibility study of enhanced CJD surveillance on the elderly was begun in 2016. Recruitment was lower than predicted. We describe a review of the challenges encountered in that study: identification, referral, and recruitment, and the effects of actions based on the results of that review. Methods Review was conducted in 2017. Study data for all eligible cases identified and referred from one participating service (Anne Rowling clinic (ARC)) was curated and anonymised in a bespoke database. A questionnaire was sent out to all the clinicians in medicine of the elderly, psychiatry of old age and neurology (including ARC) specialties in NHS Lothian, exploring possible reasons for low recruitment. Results Sixty-eight cases were referred from the ARC (March 2016-September 2017): 25% were recruited. Most cases had been referred because of diagnostic uncertainty. No difference was seen between those recruited and the non-recruited, apart from age and referrer. Twelve of 60 participating clinicians completed the questionnaire: only 4 had identified eligible cases. High workload, time constraints, forgetting to refer, unfamiliarity with the eligibility criteria, and the rarity of eligible cases, were some of the reasons given. Suggestions as to how to improve referral of eligible cases included: regular email reminders, feedback to referrers, improving awareness of the study, visible presence of the study team, and integration of the study with other research oriented services. These results were used to increase recruitment but without success. Conclusion Recruitment was lower than predicted. Actions taken following a review at 21 months did not lead to significant improvement; recruitment remained low, with many families/patients declining to take part (75%). In assessing the failure to improve recruitment, two factors need to be considered. Firstly, the initial referral rate was expected to be higher because of existing patients already known to the clinical services, with later referrals being only newly presenting patients. Secondly, the unplanned absence of a dedicated study nurse. Searching digital records/anonymised derivatives to identify eligible patients could be explored.

Abstract Background Variant Creutzfeldt-Jakob Disease (vCJD) is primarily associated with dietary exposure to bovine-spongiform-encephalopathy. Cases may be missed in the elderly population where dementia is common with less frequent referral to specialist neurological services. This study’s twin aims were to determine the feasibility of a method to detect possible missed cases in the elderly population and to identify any such cases. Methods A multi-site study was set-up in Lothian in 2016, to determine the feasibility of enhanced CJD-surveillance in the 65 + population-group, and undertake a clinicopathological investigation of patients with features of ‘atypical’ dementia. Results Thirty patients are included; 63% male, 37% female. They were referred because of at least one neurological feature regarded as ‘atypical’ (for the common dementing illnesses): cerebellar ataxia, rapid progression, or somato-sensory features. Mean-age at symptom-onset (66 years, range 53–82 years), the time between onset-of-symptoms and referral to the study (7 years, range 1–13 years), and duration-of-illness from onset-of-symptoms until death or the censor-date (9.5 years, range 1.1–17.4 years) were determined. By the censor-date, 9 cases were alive and 21 had died. Neuropathological investigations were performed on 10 cases, confirming: Alzheimer’s disease only (2 cases), mixed Alzheimer’s disease with Lewy bodies (2 cases), mixed Alzheimer’s disease with amyloid angiopathy (1 case), moderate non-amyloid small vessel angiopathy (1 case), a non-specific neurodegenerative disorder (1 case), Parkinson's disease with Lewy body dementia (1 case), and Lewy body dementia (2 cases). No prion disease cases of any type were detected. Conclusion The surveillance approach used was well received by the local clinicians and patients, though there were challenges in recruiting sufficient cases; far fewer than expected were identified, referred, and recruited. Further research is required to determine how such difficulties might be overcome. No missed cases of vCJD were found. However, there remains uncertainty whether this is because missed cases are very uncommon or because the study had insufficient power to detect them.