From the Departments of Neurology (M.B., J.R.D., J.G.-R., K.A.J.), Psychiatry and Psychology (M.M.M.), Radiology (N.T.T.P., M.L.S., C.R.J., V.J.L., J.L.W.), Health Science Research (P.R.M.), and Information Technology (M.L.S.), Mayo Clinic, Rochester, MN; and Departments of Neurology (N.E.-T.) and Neuroscience (N.E.-T., D.W.D.), Mayo Clinic, Jacksonville, FL.
Neurology. 2021 Aug 3;97(5):e523-e534. doi: 10.1212/WNL.0000000000012322. Epub 2021 Jun 4.
To assess and compare demographic, clinical, neuroimaging, and pathologic characteristics of a cohort of patients with right hemisphere-predominant vs left hemisphere-predominant logopenic progressive aphasia (LPA).
This is a case-control study of patients with LPA who were prospectively followed at Mayo Clinic and underwent [F]-fluorodeoxyglucose (FDG) PET scan. Patients were classified as rLPA if right temporal lobe metabolism was ≥1 SD lower than left temporal lobe metabolism. Patients with rLPA were frequency-matched 3:1 to typical left-predominant LPA based on degree of asymmetry and severity of temporal lobe metabolism. Patients were compared on clinical, imaging (MRI, FDG-PET, β-amyloid, and tau-PET), and pathologic characteristics.
Of 103 prospectively recruited patients with LPA, 8 (4 female) were classified as rLPA (7.8%); all patients with rLPA were right-handed. Patients with rLPA had milder aphasia based on the Western Aphasia Battery-Aphasia Quotient ( = 0.04) and less frequent phonologic errors ( = 0.015). Patients with rLPA had shorter survival compared to typical LPA: hazard ratio 4.0 (1.2-12.9), = 0.02. There were no other differences in demographics, handedness, genetics, or neurologic or neuropsychological tests. Compared to the 24 frequency-matched patients with typical LPA, patients with rLPA showed greater frontotemporal hypometabolism of the nondominant hemisphere on FDG-PET and less atrophy in amygdala and hippocampus of the dominant hemisphere. Autopsy evaluation revealed a similar distribution of pathologic findings in both groups, with Alzheimer disease pathologic changes being the most frequent pathology.
rLPA is associated with less severe aphasia but has shorter survival from reported symptom onset than typical LPA, possibly related to greater involvement of the nondominant hemisphere.
评估和比较右半球优势与左半球优势语义性进行性失语症(LPA)患者队列的人口统计学、临床、神经影像学和病理学特征。
这是一项前瞻性研究,对在 Mayo 诊所接受随访的 LPA 患者进行了病例对照研究,并进行了[F]-氟脱氧葡萄糖(FDG)PET 扫描。如果右侧颞叶代谢比左侧颞叶代谢低≥1 个标准差,则将患者归类为 rLPA。根据不对称程度和颞叶代谢严重程度,将 rLPA 患者与典型的左优势 LPA 进行频率匹配,3:1。比较患者的临床、影像学(MRI、FDG-PET、β-淀粉样蛋白和 tau-PET)和病理学特征。
在 103 例前瞻性招募的 LPA 患者中,有 8 例(4 例女性)被归类为 rLPA(7.8%);所有 rLPA 患者均为右利手。rLPA 患者的失语症程度较轻,根据西方失语症成套测验-失语症商数( = 0.04)和较少的语音错误( = 0.015)。与典型 LPA 相比,rLPA 患者的生存期更短:危险比 4.0(1.2-12.9), = 0.02。两组在人口统计学、利手、遗传学或神经学或神经心理学测试方面无其他差异。与 24 例典型 LPA 频率匹配患者相比,rLPA 患者在 FDG-PET 上表现出非优势半球的额颞叶代谢更低,而优势半球的杏仁核和海马萎缩更少。尸检评估显示两组的病理学发现分布相似,阿尔茨海默病病理改变最常见。
rLPA 与较轻微的失语症相关,但从报告的症状发作到发病的生存时间比典型 LPA 短,可能与非优势半球的更大参与有关。