Concord General Hospital, Sydney, Australia.
Brain and Mind Centre, University of Sydney Medical School, Sydney, Australia.
J Alzheimers Dis. 2018;61(2):705-715. doi: 10.3233/JAD-170743.
Adynamic speech is characteristic of progressive supranuclear palsy (PSP), but higher language deficits have been reported inconsistently, in the context of clinical and pathological overlaps with progressive non-fluent aphasia (PNFA).
The present study tested two hypotheses: 1) PSP and PNFA display impaired single word repetition, object naming, semantic knowledge, and syntactic comprehension; and 2) PSP have reduced speed on timed cognitive tasks.
Structured clinical and neuropsychological assessments of language were performed on patients with clinically defined PSP and PNFA. Language was tested using the Sydney Language Battery (SYDBAT) and the Test of Reception of Grammar (TROG).
In total, 144 participants were studied (PSP 22, PNFA 29, and Control 93). PSP patients had prominent eye movement abnormalities, parkinsonism, and falls. All 4 PSP patients who underwent postmortem examination had 4-Repeat tauopathy, with PSP pathology in 3. The frequency and severity of impairment on the SYDBAT (naming, word comprehension, semantic association), and TROG (syntactic comprehension) did not differ between PSP and PNFA, but PSP were significantly slower on timed non-language cognitive tests.
Tested formally, aphasia may be seen in PSP, with a severity similar to that seen in PNFA.
运动不能性言语是进行性核上性麻痹(PSP)的特征,但在与进行性非流利性失语症(PNFA)的临床和病理重叠的情况下,语言缺陷报道不一致。
本研究检验了两个假设:1)PSP 和 PNFA 表现出单字重复、物体命名、语义知识和句法理解受损;2)PSP 在计时认知任务上的速度降低。
对临床上定义的 PSP 和 PNFA 患者进行了结构化的临床和神经心理学语言评估。使用悉尼语言电池(SYDBAT)和语法接受测试(TROG)测试语言。
共研究了 144 名参与者(PSP 22 名,PNFA 29 名,对照组 93 名)。PSP 患者有明显的眼球运动异常、帕金森病和跌倒。所有 4 名接受尸检的 PSP 患者均有 4 重复 tau 病,其中 3 名患者有 PSP 病理学。在 SYDBAT(命名、单词理解、语义联想)和 TROG(句法理解)上的损伤频率和严重程度在 PSP 和 PNFA 之间没有差异,但 PSP 在计时非语言认知测试上的速度明显较慢。
正式测试表明,PSP 可能出现失语症,严重程度与 PNFA 相似。