Cerebellar atrophy and its contribution to cognition in frontotemporal dementias
Cerebellar atrophy and its contribution to cognition in frontotemporal dementias
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DOI：
10.1002/ana.25271
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发表时间：
2018-07-01
影响因子：
11.2
通讯作者：
Piguet, Olivier
中科院分区：
文献类型：
--
作者：
Chen, Yu;Kumfor, Fiona;Piguet, Olivier
目的越来越多的证据表明，小脑损伤对认知功能有影响。额颞性痴呆(FTD)是一种神经退行性疾病，主要影响额叶和/或颞叶。有三种主要的表型，每一种都有不同的临床和认知特征：行为变异型FTD(BvFTD)、语义性痴呆(Sd)和进行性非流利性失语症(PNFA)。然而，FTD患者小脑改变的严重程度及其与认知的关系仍不清楚。本研究旨在建立FTD亚型小脑灰质的MRI改变及其与认知功能障碍的关系。方法96例符合现行临床诊断标准的FTD患者(45例BvFTD、28例SD和23例PNFA)和35例年龄、性别、教育程度匹配的对照组进行了脑MRI和认知功能评估。结果与对照组相比，所有FTD亚型均可观察到双侧小脑广泛的改变，其中bvFTD萎缩程度最大。在bvFTD的注意力和工作记忆、SD的视觉空间功能和PNFA的语言运动功能中，小脑完整性与认知表现显著相关。无论FTD的表型如何，双侧小腿和VI小叶的萎缩最常与认知功能障碍相关。解释本研究首次在FTD综合征中发现了不同的小脑萎缩模式，在考虑了脑萎缩的影响后，小脑萎缩又与离散的认知功能障碍有关。这些发现扩展了我们对小脑的理解，并指出它参与了运动功能以外的一系列过程。Ann Neurol 2018；83：98-109
ObjectiveIncreasing evidence suggests that cerebellar damage impacts on cognitive functions. Frontotemporal dementias (FTDs) are neurodegenerative brain conditions, primarily affecting the frontal and/or temporal lobe. Three main phenotypes are recognized, each with a distinct clinical and cognitive profile: behavioral-variant FTD (bvFTD), semantic dementia (SD), and progressive nonfluent aphasia (PNFA). The severity of cerebellar changes and their relation to cognition in FTD, however, remain unclear. This study aimed to establish cerebellar gray matter changes on magnetic resonance imaging (MRI) and their relation to profiles of cognitive deficits in FTD subtypes.MethodsNinety-six FTD patients (45 bvFTD, 28 SD, and 23 PNFA), meeting current clinical diagnostic criteria, and 35 age-, sex-, and education-matched controls underwent brain MRI and cognitive assessment. Cerebral and cerebellar gray matter integrity were investigated using voxel-based morphometry.ResultsCompared with controls, widespread bilateral cerebellar changes were observed in all FTD subtypes, with the greatest atrophy present in bvFTD. Significant associations were found between cerebellar integrity and cognitive performance in attention and working memory in bvFTD, visuospatial function in SD, and language-motor function in PNFA. Bilateral atrophy of crus and lobule VI were most commonly associated with cognitive deficits, irrespective of FTD phenotype.InterpretationThis study is the first to identify distinct patterns of cerebellar atrophy across FTD syndromes, which in turn relate to discrete cognitive dysfunctions, after accounting for the effect of cerebral atrophy. These findings extend our understanding of the cerebellum and point to its involvement across an array of processes beyond the domain of motor function. Ann Neurol 2018;83:98-109