Thalamic nuclei in frontotemporal dementia: Mediodorsal nucleus involvement is universal but pulvinar atrophy is unique to C9orf72
Thalamic nuclei in frontotemporal dementia: Mediodorsal nucleus involvement is universal but pulvinar atrophy is unique to C9orf72
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DOI：
10.1002/hbm.24856
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发表时间：
2019-11-07
影响因子：
4.8
通讯作者：
Rohrer, Jonathan D.
中科院分区：
文献类型：
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作者：
Bocchetta, Martina;Iglesias, Juan E.;Rohrer, Jonathan D.
丘脑萎缩是所有形式的FTD的共同特征，但对特定的核团受累知之甚少。我们的目标是通过FTD频谱研究体内丘脑核团的萎缩。将402名FTD患者(年龄：平均(SD)64.3(8.2)岁；病程：4.8(2.8)年)与104名年龄匹配的对照组(年龄：62.5(10.4)岁)进行比较，使用T1加权磁共振成像的自动分割来提取14个丘脑核团的体积。根据临床诊断(行为变异型FTD 180例，语义型变异型原发性进行性失语(SvPPA)85例，非流利变异型PPA(NfvPPA)114例，PPA-NOS 15例，运动神经元相关疾病(FTD-MND)8例)，遗传学诊断(MAPT 27例，C9orf72 28例，GRN 18例)，病理证实(Ta病37例，TDP-43例，FU病4例)进行分层。在所有FTD亚组中，背内侧核(MD)是唯一受累的核(比对照组小16-33%)。在遗传性病例(28-38%)、TDP-43 A型(47%)、tau-CBD(44%)和FTD-MND(53%)中，背外侧核也受到特别的影响。枕骨受累仅C9orf72组(16%)。在遗传性病例中，外侧和内侧膝状核也受到影响(10-20%)，尤其是C9orf72扩张型携带者的LGN。使用单个丘脑核团体积比使用整个丘脑体积在区分FTD组方面提供了更高的准确性。MD是所有FTD组中唯一受影响的结构。在不同的FTD类型中可以看到丘脑核的不同受累，在C9orf72扩张性携带者中，枕部有一种独特的萎缩模式。
Thalamic atrophy is a common feature across all forms of FTD but little is known about specific nuclei involvement. We aimed to investigate in vivo atrophy of the thalamic nuclei across the FTD spectrum. A cohort of 402 FTD patients (age: mean(SD) 64.3(8.2) years; disease duration: 4.8(2.8) years) was compared with 104 age-matched controls (age: 62.5(10.4) years), using an automated segmentation of T1-weighted MRIs to extract volumes of 14 thalamic nuclei. Stratification was performed by clinical diagnosis (180 behavioural variant FTD (bvFTD), 85 semantic variant primary progressive aphasia (svPPA), 114 nonfluent variant PPA (nfvPPA), 15 PPA not otherwise specified (PPA-NOS), and 8 with associated motor neurone disease (FTD-MND), genetic diagnosis (27 MAPT, 28 C9orf72, 18 GRN), and pathological confirmation (37 tauopathy, 38 TDP-43opathy, 4 FUSopathy). The mediodorsal nucleus (MD) was the only nucleus affected in all FTD subgroups (16-33% smaller than controls). The laterodorsal nucleus was also particularly affected in genetic cases (28-38%), TDP-43 type A (47%), tau-CBD (44%), and FTD-MND (53%). The pulvinar was affected only in the C9orf72 group (16%). Both the lateral and medial geniculate nuclei were also affected in the genetic cases (10-20%), particularly the LGN in C9orf72 expansion carriers. Use of individual thalamic nuclei volumes provided higher accuracy in discriminating between FTD groups than the whole thalamic volume. The MD is the only structure affected across all FTD groups. Differential involvement of the thalamic nuclei among FTD forms is seen, with a unique pattern of atrophy in the pulvinar in C9orf72 expansion carriers.