Department of Neurology, Center for Movement Disorders and Neuromodulation, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany.
PLoS One. 2012;7(7):e41873. doi: 10.1371/journal.pone.0041873. Epub 2012 Jul 27.
BACKGROUND/OBJECTIVE: Corticobasal syndrome (CBS) is a rare neurodegenerative disorder characterized by a progressive and asymmetric manifestation of cortical and basal-ganglia symptoms of different origin. The spatio-temporal dynamics of cerebral atrophy in CBS is barely known. This study aimed to longitudinally quantify the individual dynamics of brain volume changes in patients with CBS as compared to healthy controls.
We used deformation-field-based morphometry (DFM) to study volumetric changes of each individual brain in short intervals of a few months. DFM enabled the quantitative analysis of local volume changes without predefining regions of interest in MR images of 6 patients with CBS and 11 matched healthy controls. A total of 64 whole brain 3D-MR-scans were acquired two to eight times over periods of 14 to 26 months. Based on repeated registrations of MR observations to the initial scan, maps of local volume ratio changes were computed.
Compared to controls patients showed significant and increasing volume loss over time in premotor and primary-motor-cortices, somatosensory area 3a, superior parietal areas BA 5/7, and corticospinal tract. Furthermore, significant and asymmetric atrophy was identified in the caudate nucleus head, putamen, pallidum, motor-thalamus and substantia nigra. Temporal lobe was affected in those patients who presented progressive cognitive impairment.
The analysis revealed localized, pathological changes in brains of patients with CBS, which differed significantly from those occurring during aging in healthy controls. As compared to age- and sex-matched controls, brains of CBS patients showed a common degenerating neural network comprising the motor circuit with basal ganglia and motor thalamic nuclei as well as the premotor and primary-motor-cortex.
背景/目的:皮质基底节综合征(CBS)是一种罕见的神经退行性疾病,其特征为皮质和基底节症状的进行性和不对称表现,而这些症状具有不同的起源。皮质基底节综合征患者脑萎缩的时空动态变化目前知之甚少。本研究旨在纵向定量比较皮质基底节综合征患者与健康对照者的脑容量变化的个体动态。
我们使用基于变形场的形态计量学(DFM)在数月的短时间内研究每个个体大脑的体积变化。DFM 使我们能够在不预先定义磁共振图像中感兴趣区域的情况下,对患者 6 例和健康对照者 11 例的脑进行局部体积变化的定量分析。在 14 至 26 个月的时间内,共获得 64 例全脑 3D-MR 扫描,扫描次数为 2 至 8 次。基于对初始扫描的磁共振观察的重复配准,计算了局部体积比变化图。
与对照组相比,患者的大脑在运动前皮质和初级运动皮质、体感区 3a、顶叶上区 BA5/7 和皮质脊髓束中随时间推移显示出显著且不断增加的体积损失。此外,在尾状核头部、壳核、苍白球、运动丘脑和黑质中也发现了显著的不对称性萎缩。在那些出现进行性认知障碍的患者中,颞叶受到了影响。
该分析揭示了皮质基底节综合征患者大脑中的局部病理性变化,与健康对照组中发生的衰老过程明显不同。与年龄和性别匹配的对照组相比,皮质基底节综合征患者的大脑显示出一个共同的退行性神经网络,包括运动回路和基底节及运动丘脑核,以及运动前皮质和初级运动皮质。