Progression of brain atrophy in PSP and CBS over 6 months and 1 year
Progression of brain atrophy in PSP and CBS over 6 months and 1 year
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DOI：
10.1212/wnl.0000000000003305
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发表时间：
2016-11-08
期刊：
影响因子：
9.9
通讯作者：
Boxer, Adam L.
中科院分区：
文献类型：
--
作者：
Dutt, Shubir;Binney, Richard J.;Boxer, Adam L.
目的：检查体积MRI在测量4种重复调节性疾病进展中的实用性和可靠性在55例PSP患者和33例CBS患者（78％淀粉样蛋白PET阴性）中，研究了区域MRI体积和PSP评级量表评分的12个月变化，而多中心自然历史研究中的30例正常对照组进行了检查。基于纵素体素的形态计量学分析确定了体积损失的模式，利益区域分析检查了基于先前经过验证的Atlases，基于脑干（中脑，中脑，小脑梗），皮层和皮层下区域的体积损失率。将结果与226例PSP患者的复制队列中的结果进行了比较PSP;但是，中脑和桥脑萎缩率相似。与正常对照相比，体素分析在每组中显示出区域纵向萎缩的不同模式。诊断之间的中脑/PON体积比有所不同，但随着时间的推移保持稳定。在两个患者组中，脑干萎缩率与使用PSP评级量表测量的疾病进展相关。结论：在CBS和PSP中，在6个月内，体积损失是可量化的。未来的临床试验可能能够结合CBS和PSP来测量治疗作用。
Objective: To examine the utility and reliability of volumetric MRI in measuring disease progression in the 4 repeat tauopathies, progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), to support clinical development of new tau-directed therapeutic agents.Methods: Six- and 12-month changes in regional MRI volumes and PSP Rating Scale scores were examined in 55 patients with PSP and 33 patients with CBS (78% amyloid PET negative) compared to 30 normal controls from a multicenter natural history study. Longitudinal voxel-based morphometric analyses identified patterns of volume loss, and region-of-interest analyses examined rates of volume loss in brainstem (midbrain, pons, superior cerebellar peduncle), cortical, and subcortical regions based on previously validated atlases. Results were compared to those in a replication cohort of 226 patients with PSP with MRI data from the AL-108-231 clinical trial.Results: Patients with CBS exhibited greater baseline atrophy and greater longitudinal atrophy rates in cortical and basal ganglia regions than patients with PSP; however, midbrain and pontine atrophy rates were similar. Voxel-wise analyses showed distinct patterns of regional longitudinal atrophy in each group as compared to normal controls. The midbrain/pons volumetric ratio differed between diagnoses but remained stable over time. In both patient groups, brainstem atrophy rates were correlated with disease progression measured using the PSP Rating Scale.Conclusions: Volume loss is quantifiable over a period of 6 months in CBS and PSP. Future clinical trials may be able to combine CBS and PSP to measure therapeutic effects.