From the Division of Speech Pathology (J.R.D.), Department of Neurology (P.T., K.A.J., R.S.) and Departments of Radiology (J.L.W.), Psychiatry and Psychology (Neuropsychology) (M.M.M.), and Health Sciences Research (A.M.), Mayo Clinic, Rochester, MN.
Neurology. 2024 Aug 27;103(4):e209693. doi: 10.1212/WNL.0000000000209693. Epub 2024 Jul 30.
No epidemiologic studies have formally assessed the incidence of primary progressive aphasia (PPA) and primary progressive apraxia of speech (PPAOS). Thus, we decided to assess the incidence of these disorders in Olmsted County, MN, between 2011 and 2022, and to characterize clinical, radiographic, and pathologic characteristics of these patients.
This was a retrospective examination of data from a population-based cohort of patients with PPA and PPAOS prospectively identified in Olmsted County, MN, from 2011 to 2022. The incidence of PPA among adults (older than 18 years) was calculated for Olmsted County as the number of patients per 100,000 person-years during the study period. The adult population of Olmsted County was determined by the annual catchment population reported by the Rochester Epidemiological Project for each year 2011-2022. A behavioral neurologist verified the clinical diagnoses and determined subtypes.
We identified 10 patients (60% female) within the study period (median age of symptoms onset: 70 years; range: 66-73), 8 with PPA and 2 with PPAOS. Of the 8 patients with PPA (6 female patients, 2 male patients), 2 met criteria for non-fluent variant PPA (nfvPPA), 3 for logopenic variant PPA (lvPPA), and 3 for semantic variant (svPPA). Speech evaluation confirmed the clinical diagnoses in all patients and all showed typical imaging findings consistent with their respective subtype. Six patients (2 PPAOS, 2 nfvPPA, 2 lvPPA) died and 3 underwent autopsy (2 PPAOS, 1 nfvPPA), confirming the pathologic diagnosis of progressive supranuclear palsy. The incidence of PPA + PPAOS was 0.70 persons per 100,000 person-years (95% CI 0.34-1.29 persons per 100,000) during the study period. The incidence of PPAOS was 0.14 persons per 100,000 person-years (95% CI 0.02-0.55 persons per 100,000), whereas for the 8 patients with PPA, the incidence was 0.56 persons per 100,000 person-years (95% CI 0.24-1.10 cases per 100,000). The incidence of nfvPPA was 0.14 persons per 100,000 person-years (95% CI 0.02-0.55), 0.21 persons per 100,000 person-years (95% CI 0.04-0.61) for lvPPA, and 0.21 persons per 100,000 person-years (95% CI 0.04-0.61) for svPPA.
As a group, PPA and PPAOS are a relatively rare group of diseases. PPAOS has a slightly lower incidence than PPA as a group but similar incidence to the individual PPA variants.
目前尚无流行病学研究正式评估原发性进行性失语症（PPA）和原发性进行性构音障碍（PPAOS）的发病率。因此，我们决定评估明尼苏达州奥姆斯特德县在 2011 年至 2022 年期间这些疾病的发病率，并对这些患者的临床、影像学和病理学特征进行描述。
这是一项对奥姆斯特德县前瞻性确定的 PPA 和 PPAOS 患者的基于人群队列数据的回顾性研究，时间为 2011 年至 2022 年。奥姆斯特德县成年人（年龄大于 18 岁）的 PPA 发病率按每 10 万人年的患者数计算。奥姆斯特德县的成年人口由罗切斯特流行病学项目每年报告的年度集水区人口确定。一名行为神经病学家对临床诊断进行了核实，并确定了亚型。
在研究期间，我们共发现了 10 例患者（60%为女性）（症状发作的中位年龄：70 岁；范围：66-73 岁），其中 8 例为 PPA，2 例为 PPAOS。在 8 例 PPA 患者中（6 例为女性患者，2 例为男性患者），2 例符合非流利型变异 PPA（nfvPPA）标准，3 例符合 logopenic 变异 PPA（lvPPA）标准，3 例符合语义变异（svPPA）标准。言语评估在所有患者中均确认了临床诊断，所有患者均显示出与各自亚型一致的典型影像学表现。6 例患者（2 例 PPAOS，2 例 nfvPPA，2 例 lvPPA）死亡，3 例接受了尸检（2 例 PPAOS，1 例 nfvPPA），证实了进行性核上性麻痹的病理学诊断。PPA+PPAOS 的发病率为每 10 万人年 0.70 人（95%CI：0.34-1.29 人/10 万人年）。PPAOS 的发病率为每 10 万人年 0.14 人（95%CI：0.02-0.55 人/10 万人年），而 8 例 PPA 患者的发病率为每 10 万人年 0.56 人（95%CI：0.24-1.10 例/10 万人年）。nfvPPA 的发病率为每 10 万人年 0.14 人（95%CI：0.02-0.55），lvPPA 的发病率为每 10 万人年 0.21 人（95%CI：0.04-0.61），svPPA 的发病率为每 10 万人年 0.21 人（95%CI：0.04-0.61）。
作为一个整体，PPA 和 PPAOS 是一组相对罕见的疾病。PPAOS 的发病率略低于 PPA，但与个别 PPA 变异的发病率相似。
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