TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia
TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia
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DOI：
10.1212/wnl.0b013e318202038c
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发表时间：
2010-12-14
期刊：
影响因子：
9.9
通讯作者：
Seeley, W. W.
中科院分区：
文献类型：
--
作者：
Rohrer, J. D.;Geser, F.;Seeley, W. W.
背景资料：我们试图描述与TDP-43免疫反应性inclusions（FTLD-TDP）的额颞叶变性患者的生前临床和神经影像学特征。方法：受试者被招募从一个连续的一系列患者的主要神经病理诊断FTLD-TDP和生前MRI。28例患者符合入选标准：9例为1型，5例为2型，10例为3型FTLD-TDP。4例患者的FTLD-TDP病理过于稀疏，无法进行亚型分型。我们回顾了这些病例的临床、神经心理学和神经影像学特征。体素为基础的形态测量被用来评估区域灰质萎缩的一组50认知正常对照subjects.Results：临床诊断不同的群体之间：语义性痴呆只与1型病理，而进行性非流利性失语症和皮质基底综合征只与3型。行为变异型额颞叶痴呆和额颞叶痴呆伴运动神经元病见于2型或3型病理。神经影像学分析显示不同病理亚型的萎缩模式不同：1型与不对称性前颞叶萎缩相关2型：颞叶内侧、前额叶内侧和眶额-岛叶皮质相对对称性萎缩;第三型为不对称性萎缩（以左侧或右侧为主），累及更多背侧区域，包括额叶、颞叶和顶叶下皮质以及纹状体和丘脑。没有显着萎缩的患者过于稀疏的病理亚型。结论：FTLD-TDP亚型有不同的临床和神经影像学特征，突出FTLD-TDP亚型的临床病理相关性的相关性。神经病学（R）2010;75：2204-2211
Background: We sought to describe the antemortem clinical and neuroimaging features among patients with frontotemporal lobar degeneration with TDP-43 immunoreactive inclusions (FTLD-TDP).Methods: Subjects were recruited from a consecutive series of patients with a primary neuropathologic diagnosis of FTLD-TDP and antemortem MRI. Twenty-eight patients met entry criteria: 9 with type 1, 5 with type 2, and 10 with type 3 FTLD-TDP. Four patients had too sparse FTLD-TDP pathology to be subtyped. Clinical, neuropsychological, and neuroimaging features of these cases were reviewed. Voxel-based morphometry was used to assess regional gray matter atrophy in relation to a group of 50 cognitively normal control subjects.Results: Clinical diagnosis varied between the groups: semantic dementia was only associated with type 1 pathology, whereas progressive nonfluent aphasia and corticobasal syndrome were only associated with type 3. Behavioral variant frontotemporal dementia and frontotemporal dementia with motor neuron disease were seen in type 2 or type 3 pathology. The neuroimaging analysis revealed distinct patterns of atrophy between the pathologic subtypes: type 1 was associated with asymmetric anterior temporal lobe atrophy (either left-or right-predominant) with involvement also of the orbitofrontal lobes and insulae; type 2 with relatively symmetric atrophy of the medial temporal, medial prefrontal, and orbitofrontal-insular cortices; and type 3 with asymmetric atrophy (either left-or right-predominant) involving more dorsal areas including frontal, temporal, and inferior parietal cortices as well as striatum and thalamus. No significant atrophy was seen among patients with too sparse pathology to be subtyped.Conclusions: FTLD-TDP subtypes have distinct clinical and neuroimaging features, highlighting the relevance of FTLD-TDP subtyping to clinicopathologic correlation. Neurology (R) 2010;75:2204-2211