From the Banner Sun Health Research Institute (BND, MNS, HAS, SJ, CB, KJD, LIS, TGB), Sun City; and Mayo Clinic (BND, JGH, CHA, JNC, ED-D), Scottsdale, Arizona.
J Neuropathol Exp Neurol. 2014 Mar;73(3):244-52. doi: 10.1097/NEN.0000000000000046.
Existing reports on the frequencies of neurodegenerative diseases are typically based on clinical diagnoses. We sought to determine these frequencies in a prospectively assessed, community-based autopsy series. Included subjects had normal cognitive and movement disorder assessments at study entry. Of the 119 cases meeting these criteria, 52% were women; the median age of study entry was 83.5 years (range, 67-99 years), and the median duration from the first visit until death was 4.3 years (range, 0-10 years). At autopsy, clinicopathological diagnoses were made in 30 cases (25%). These diagnoses included 20 with Alzheimer disease (AD) (17%), 7 with vascular dementia (6%), 4 with progressive supranuclear palsy (3%), 3 with Parkinson disease and 1 each with dementia with Lewy bodies, corticobasal degeneration, or multiple system atrophy (0.8% each). Of the 87 subjects still clinically normal at death (73%), 33 had extensive AD pathology (preclinical AD) (38%), 17 had incidental Lewy bodies (20%), and 4 had incidental pathology consistent with progressive supranuclear palsy (5%). The diagnoses were not mutually exclusive. Although limited by a relatively small sample size, the neuropathological outcome of these initially normal elderly subjects represents a rough estimate of the incidence of these neurodegenerative conditions over a defined time period.
现有的神经退行性疾病频率报告通常基于临床诊断。我们旨在通过前瞻性评估、基于社区的尸检系列来确定这些频率。纳入的研究对象在研究开始时具有正常的认知和运动障碍评估。在符合这些标准的 119 例病例中,女性占 52%;研究开始时的中位年龄为 83.5 岁(范围,67-99 岁),从首次就诊到死亡的中位时间为 4.3 年(范围,0-10 年)。尸检时,在 30 例(25%)中做出了临床病理诊断。这些诊断包括 20 例阿尔茨海默病(AD)(17%)、7 例血管性痴呆(6%)、4 例进行性核上性麻痹(3%)、3 例帕金森病和 1 例路易体痴呆、皮质基底节变性或多系统萎缩(各占 0.8%)。在死亡时仍处于临床正常状态的 87 例(73%)中,33 例有广泛的 AD 病理学(临床前 AD)(38%),17 例有偶然的路易体(20%),4 例有偶然的符合进行性核上性麻痹的病理学(5%)。这些诊断并不相互排斥。尽管受到样本量相对较小的限制,但这些最初正常的老年患者的神经病理学结果代表了在定义时间段内这些神经退行性疾病发生率的大致估计。