Whitwell Jennifer L, Martin Peter, Duffy Joseph R, Clark Heather M, Utianski Rene L, Botha Hugo, Machulda Mary M, Strand Edythe A, Josephs Keith A
Department of Radiology (JLW), Department of Health Sciences Research (PM), Division of Speech Pathology (JRD, HMC, RLU, EAS), and Division of Behavioral Neurology (HB, KAJ), Department of Neurology, and Department of Psychiatry and Psychology (MMM), Mayo Clinic, Rochester, MN.
Neurol Clin Pract. 2021 Jun;11(3):249-255. doi: 10.1212/CPJ.0000000000000919.
To compare survival among patients with different combinations of apraxia of speech (AOS) and agrammatic aphasia, including those with isolated AOS (primary progressive AOS, PPAOS), both AOS and agrammatic aphasia (AOS + progressive agrammatic aphasia [PAA]), and isolated agrammatic aphasia (PAA).
One hundred nine patients were recruited who had any combination of AOS and agrammatic aphasia (42 PPAOS, 56 AOS + PAA, and 11 PAA) and were followed longitudinally, with 57 patients having since died. Cox proportional hazard models were used to quantify the relative risk of death across diagnoses. Adjusted survival curves are presented based on this model. We also assessed the influence of AOS and aphasia severity on survival.
PPAOS had the longest survival (median survival of 5.97 years from the baseline visit), followed by PAA (5.26 years) and then AOS + PAA (4.33 years). AOS + PAA had a greater risk of death than PPAOS, with a hazard ratio of 3.01 (lower/upper confidence interval = 1.66/5.46, < 0.001). Risk of death did not differ between PAA and the other groups. All results accounted for age and time from onset to baseline visit. AOS severity, independent of syndromic diagnosis, was associated with greater risk of death, with a hazard ratio of 1.35 for a 1-point increase in severity. Aphasia severity was not associated with risk of death.
Individuals with PPAOS have better survival and reduced risk of death compared with individuals with AOS + PAA. This finding will help improve prognostic estimates for these patients and supports the value of distinguishing PPAOS from AOS + PAA.
比较言语失用症（AOS）和语法性失语症不同组合患者的生存率，包括孤立性AOS（原发性进行性AOS，PPAOS）、AOS和语法性失语症两者并存（AOS + 进行性语法性失语症 [PAA]）以及孤立性语法性失语症（PAA）患者。
招募了109例患有AOS和语法性失语症任意组合的患者（42例PPAOS、56例AOS + PAA和11例PAA），并对其进行纵向随访，其中57例患者已死亡。使用Cox比例风险模型来量化不同诊断下的相对死亡风险。基于该模型呈现调整后的生存曲线。我们还评估了AOS和失语症严重程度对生存的影响。
PPAOS的生存期最长（从基线访视起中位生存期为5.97年），其次是PAA（5.26年），然后是AOS + PAA（4.33年）。AOS + PAA的死亡风险高于PPAOS，风险比为3.01（置信区间下限/上限 = 1.66/5.46，P < 0.001）。PAA与其他组之间的死亡风险没有差异。所有结果均考虑了年龄和从发病到基线访视的时间。独立于综合征诊断的AOS严重程度与更高的死亡风险相关，严重程度每增加1分，风险比为1.35。失语症严重程度与死亡风险无关。
与AOS + PAA患者相比，PPAOS患者生存期更长且死亡风险降低。这一发现将有助于改善对这些患者的预后估计，并支持将PPAOS与AOS + PAA区分开来的价值。
