From the Department of Neurology (D.P.G.-G., H.B., R.L.U., J.R.D., H.C., G.M., K.A.J.), and Department of Psychiatry and Psychology (M.M.M.), Mayo Clinic, Rochester, MN; Department of Neuroscience (Neuropathology) (D.W.D.), Mayo Clinic, Jacksonville, FL; and Department of Radiology (N.T.T.P., J.L.W.), Mayo Clinic, Rochester, MN.
Neurology. 2024 Aug 27;103(4):e209717. doi: 10.1212/WNL.0000000000209717. Epub 2024 Jul 30.
Nonverbal oral apraxia (NVOA) is the inability to plan, sequence, and execute voluntary oromotor movements in the absence of weakness. In the context of neurodegenerative disease, it remains unclear whether it is linked to a specific underlying pathologic, clinical, or neuroimaging finding. Thus, we aimed to assess the clinicopathologic and neuroimaging associations of NVOA.
We conducted a retrospective study of autopsy-confirmed patients previously assessed through an NVOA evaluation tool with a previously published cutpoint to screen for NVOA. We compared demographic and clinical characteristics and postmortem pathology between those who developed NVOA and those who did not. We also compared clinicopathologic characteristics in mild vs greater than mild NVOA and early vs late-emerging NVOA. SPM12 was used to assess patterns of gray matter loss in NVOA vs non-NVOA with age and sex included as covariates.
A total of 104 patients (median age at symptom onset 63 years, 43% female) were included in the study. 63 (60.6%) developed NVOA. NVOA appeared at a median of 4.3 years from symptom onset. 29% developed NVOA within the first 3 years. Primary progressive apraxia of speech and the nonfluent variant of primary progressive aphasia were the most common baseline diagnoses in the NVOA group while progressive supranuclear palsy (PSP) syndrome and logopenic progressive aphasia (LPA) were the most common in patients without NVOA. Atrophy of the left lateral and medial posterior frontal cortex was related to NVOA. The most common pathologies associated with NVOA were PSP (36.5%) and corticobasal degeneration (CBD) (33.3%). In patients without NVOA, PSP (26.8%) and other pathologies (26.8%) were the most frequent. 11% of patients with NVOA had persistently mild NVOA and were more likely to have baseline diagnoses of LPA, PSP syndrome, or semantic dementia. The most frequent pathologies in this group were Alzheimer disease and PSP. The pathologic associations of greater than mild NVOA were CBD and PSP.
NVOA is present in several clinical syndromes. It is most associated with PSP and CBD. NVOA is a manifestation of left lateral and medial posterior frontal cortex damage rather than a particular pathology.
非言语性口头失用症（NVOA）是指在无肌无力的情况下，无法规划、排序和执行自愿的口部运动。在神经退行性疾病的背景下，目前尚不清楚它是否与特定的潜在病理、临床或神经影像学表现有关。因此，我们旨在评估 NVOA 的临床病理和神经影像学相关性。
我们对先前通过 NVOA 评估工具进行评估并使用先前发表的切点筛查 NVOA 的经尸检证实的患者进行了回顾性研究。我们比较了出现 NVOA 与未出现 NVOA 的患者的人口统计学和临床特征以及死后病理学。我们还比较了轻度 NVOA 与重度 NVOA、早期出现 NVOA 与晚期出现 NVOA 的临床病理特征。使用 SPM12 来评估 NVOA 与非 NVOA 患者的灰质丢失模式，年龄和性别作为协变量。
共有 104 名患者（症状发作的中位年龄为 63 岁，43%为女性）纳入研究。63 名（60.6%）患者出现 NVOA。NVOA 出现在症状发作后的中位时间为 4.3 年。29%的患者在最初 3 年内出现 NVOA。原发性进行性构音障碍和非流利型原发性进行性失语症是 NVOA 组中最常见的基线诊断，而进行性核上性麻痹（PSP）综合征和失语法性进行性失语症（LPA）则是无 NVOA 患者中最常见的诊断。左侧外侧和内侧后额皮质的萎缩与 NVOA 有关。与 NVOA 最相关的病理类型是 PSP（36.5%）和皮质基底节变性（CBD）（33.3%）。在无 NVOA 的患者中，PSP（26.8%）和其他病理类型（26.8%）最常见。11%的 NVOA 患者持续存在轻度 NVOA，更可能有 LPA、PSP 综合征或语义性痴呆的基线诊断。该组中最常见的病理类型是阿尔茨海默病和 PSP。大于轻度 NVOA 的病理关联是 CBD 和 PSP。
NVOA 存在于几种临床综合征中。它与 PSP 和 CBD 最相关。NVOA 是左侧外侧和内侧后额皮质损伤的表现，而不是特定的病理学表现。
