National CJD Research & Surveillance Unit (NCJDRSU), University of Edinburgh, Western General Hospital, Edinburgh, Scotland.
NHS Lothian and Queen Margaret University, Edinburgh, Scotland.
BMC Geriatr. 2022 Jul 20;22(1):603. doi: 10.1186/s12877-022-03280-4.
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.
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.
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.
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.
变异型克雅氏病（vCJD）主要与食用含有牛海绵状脑病的食物有关。在痴呆症较为常见且很少向神经科专家服务机构转诊的老年人群中，可能会遗漏病例。本研究的双重目的是确定一种在老年人群中检测可能遗漏病例的方法的可行性，并确定是否存在此类病例。
2016 年在洛锡安区建立了一项多站点研究，以确定在 65 岁及以上人群中加强 CJD 监测的可行性，并对具有“非典型”痴呆特征的患者进行临床病理研究。
共纳入 30 例患者；男性 63%，女性 37%。他们因至少一项被认为是“非典型”的神经学特征而被转诊：小脑共济失调、快速进展或躯体感觉特征。症状发作时的平均年龄（66 岁，范围 53-82 岁）、症状发作到研究转诊的时间（7 年，范围 1-13 年）以及从症状发作到死亡或截止日期的病程（9.5 年，范围 1.1-17.4 年）。截止日期时，9 例存活，21 例死亡。对 10 例进行了神经病理学检查，证实：阿尔茨海默病（2 例）、阿尔茨海默病合并路易体（2 例）、阿尔茨海默病合并淀粉样血管病（1 例）、非淀粉样小血管血管病（1 例）、非特异性神经退行性疾病（1 例）、帕金森病伴路易体痴呆（1 例）和路易体痴呆（2 例）。未发现任何类型的朊病毒病病例。
所使用的监测方法受到当地临床医生和患者的欢迎，但在招募足够数量的病例方面存在挑战；招募的病例远少于预期。需要进一步研究以确定如何克服这些困难。未发现变异型克雅氏病的遗漏病例。然而，仍然不确定这是因为遗漏病例非常罕见，还是因为研究的力量不足以发现它们。
