Tippett Donna C, Thompson Carol B, Demsky Cornelia, Sebastian Rajani, Wright Amy, Hillis Argye E
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States of America.
Department of Physical Medicine and Rehabilitation, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States of America.
PLoS One. 2017 Aug 16;12(8):e0183212. doi: 10.1371/journal.pone.0183212. eCollection 2017.
Behavioral assessment has been investigated in frontotemporal lobar degeneration and Alzheimer's disease, but has not been explored extensively in subtypes of primary progressive aphasia (PPA). We explored the ability of a modified version of the Frontal Behavioral Inventory (FBI-mod) to discriminate between patients with distinct subtypes of PPA and patients with mild cognitive impairment (MCI). We hypothesized that individuals with nonfluent agrammatic PPA (nfaPPA) would have higher negative behavior scores than other groups and that individuals with semantic variant PPA (svPPA) would have higher disinhibition scores than other groups. Family members and/or caregivers of 120 individuals with PPA and MCI (mean age 69.54+8.75 years; 65 (54%) female; education 16.06±2.68 years; disease duration 46.47±34.26 months) completed the FBI-mod [logopenic PPA (lvPPA) n = 40. nfaPPA n = 29, svPPA n = 27, MCI n = 24]. The groups were not significantly different in age, gender, education, or disease duration. There were no significant differences between the groups for negative behaviors (p = 0.72) and disinhibition scores (p = 0.14). When comparing negative and disinhibition scores (in percent), negative scores were significantly higher in all groups (p < 0.001). When comparing subtest items, there was a pairwise difference between lvPPA and svPPA for restlessness (lvPPA < svPPA, p = 0.02, after adjusting for multiple between-group comparisons). There was a significant difference in the proportion of severe neglect between the groups with lvPPA having a lower proportion than the other two variants (p = 0.05), and there was a significant difference in the proportion of severe poor judgment between the groups with lvPPA also having a lower proportion than nfaPPA (p = 0.04). This study reveals the greater negative behavioral disturbance than disinhibition in the PPA and MCI groups of similar age and duration since onset and identifies different profiles for some specific behaviors for the PPA groups. These findings may have clinical and practical implications.
行为评估已在额颞叶变性和阿尔茨海默病中展开研究,但在原发性进行性失语(PPA)的亚型中尚未得到广泛探索。我们探究了改良版额叶行为量表(FBI-mod)区分不同PPA亚型患者与轻度认知障碍(MCI)患者的能力。我们假设非流利性语法缺失型PPA(nfaPPA)个体的负面行为得分高于其他组,且语义变异型PPA(svPPA)个体的脱抑制得分高于其他组。120名PPA和MCI患者的家庭成员和/或照料者(平均年龄69.54±8.75岁;65名(54%)为女性;受教育年限16.06±2.68年;病程46.47±34.26个月)完成了FBI-mod[语法性错语型PPA(lvPPA)n = 40,nfaPPA n = 29,svPPA n = 27,MCI n = 24]。这些组在年龄、性别、受教育程度或病程方面无显著差异。各组在负面行为(p = 0.72)和脱抑制得分(p = 0.14)方面无显著差异。比较负面和脱抑制得分(以百分比计)时,所有组的负面得分均显著更高(p < 0.001)。比较各子测试项目时,lvPPA和svPPA在坐立不安方面存在两两差异(lvPPA < svPPA,p = 0.02,在调整组间多重比较后)。lvPPA组的严重忽视比例低于其他两种变异型组(p = 0.05),且lvPPA组的严重判断不良比例也低于nfaPPA组(p = 0.04),两组之间存在显著差异。本研究揭示了在年龄和起病病程相似的PPA和MCI组中,负面行为障碍比脱抑制更为严重,并确定了PPA组某些特定行为的不同特征。这些发现可能具有临床和实际意义。