Introduction Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive neurodegenerative disease with public health implications. Mean age of onset is 68 years. Age-specific incidence declines after 80 years. This may arise from under-ascertainment or other biological features of the disease. Accurate characterisation of late-onset sCJD is important for early diagnosis, avoiding unnecessary investigations and improving ascertainment for public health purposes. Objective To phenotype the clinical features and investigation profile of sCJD in adults >80 years. Methods We analysed all probable and definite sCJD cases identified by the UK National CJD Research & Surveillance Unit over a 10-year period (2011–2021). Individuals were grouped by age of onset. Clinical features and investigation profiles were compared. Results 10.3% (123/1196) had an age of onset over 80. Median survival was shorter (3.2 vs 4.3 months; P < 0.001). Pyramidal signs (48.3% vs 34.2%; P = 0.008) and akinetic mutism (55.1% vs 33.2%; P < 0.001) were more frequent. Psychiatric symptoms (26.3% vs 39.6%; P = 0.01) and cerebellar signs (65.4% vs 78.6%, P = 0.007) were less frequent. Cognitive impairment and myoclonus were highly prevalent regardless of age. Between age groups, the diagnostic sensitivity of cerebrospinal fluid real-time quaking-induced conversion (CSF RT-QuIC) (92.9% vs 91.9%, P = 0.74) was comparable, electroencephalography was superior (41.5% vs 25.4%; P = 0.006) and MRI was inferior (67.8% vs 91.4%; P < 0.001). Conclusions Late-onset sCJD has distinct clinical features, shorter survival and a different profile of investigation sensitivity. CSF RT-QuIC, MRI brain and specialist CJD review is recommended in older adults with a rapidly progressive neurological disorder. Autopsy is valuable when the cause remains elusive.

中文翻译：

---

80 岁以上成人散发性克雅氏病：英国监测的 10 年回顾

简介 散发性克雅氏病 (sCJD) 是一种快速进展的神经退行性疾病，具有公共卫生影响。平均发病年龄为 68 岁。 80 岁后特定年龄发病率下降。这可能是由于对该疾病的确定不足或其他生物学特征造成的。晚发性克雅氏病的准确特征对于早期诊断、避免不必要的调查和改善公共卫生目的的确定非常重要。目的 分析 80 岁以上成人 sCJD 的临床特征和调查概况。方法 我们分析了英国国家克雅氏病研究和监测部门在 10 年期间（2011 年至 2021 年）发现的所有可能和明确的 sCJD 病例。个体按发病年龄分组。比较临床特征和研究概况。结果 10.3% (123/1196) 的发病年龄超过 80 岁。中位生存期较短（3.2 个月 vs 4.3 个月；P < 0.001）。锥体征（48.3% vs 34.2%；P = 0.008）和运动不能性沉默症（55.1% vs 33.2%；P < 0.001）更为常见。精神症状（26.3% vs 39.6%；P = 0.01）和小脑体征（65.4% vs 78.6%，P = 0.007）频率较低。无论年龄大小，认知障碍和肌阵挛都很普遍。不同年龄组之间，脑脊液实时震动诱导转换（CSF RT-QuIC）的诊断灵敏度（92.9% vs 91.9%，P = 0.74）相当，脑电图诊断灵敏度更高（41.5% vs 25.4%；P = 0.006） ），MRI 较差（67.8% vs 91.4%；P < 0.001）。结论 晚发 sCJD 具有独特的临床特征、较短的生存期和不同的研究敏感性。建议患有快速进展性神经系统疾病的老年人进行脑脊液 RT-QuIC、脑部 MRI 和专家 CJD 审查。当原因仍然难以捉摸时，尸检是有价值的。