Department of Neurology and Neuropsychology, and CMMR PACA Ouest, CHU Timone, Assistance Publique-Hôpitaux de Marseille, Marseille, France.
Department of Nuclear Medecine, CHU Timone, Assistance Publique-Hôpitaux de Marseille, Marseille, France.
J Alzheimers Dis. 2020;74(1):331-343. doi: 10.3233/JAD-190961.
Corticobasal syndrome (CBS) is a neuropathologically heterogeneous entity. The use of cerebrospinal fluid and amyloid biomarkers enables detection of underlying Alzheimer's disease (AD) pathology. We thus compared clinical, eye movement, and 18FDG-PET imaging characteristics in CBS in two groups of patients divided according to their amyloid biomarkers profile. Fourteen patients presenting with CBS and amyloidosis (CBS-A+) were compared with 16 CBS patients without amyloidosis (CBS-A-). The two groups showed similar motor abnormalities (parkinsonism, dystonia) and global cognitive functions. Unlike CBS-A+ patients who displayed more posterior cortical abnormalities, CBS-A- patients demonstrated more anterior cortical and brain stem dysfunctions on the basis of neuropsychological testing, study of saccade velocities and brain hypometabolism areas on 18FDG-PET. Interestingly, Dopamine Transporter SPECT imaging showed similar levels of dopaminergic degeneration in both groups. These findings confirm common and distinct brain abnormalities between the different neurodegenerative diseases that result in CBS. We demonstrate the importance of a multidisciplinary approach to improve diagnosis in vivo in particular on oculomotor examination.
皮质基底节综合征（CBS）是一种神经病理学异质性实体。脑脊液和淀粉样蛋白生物标志物的使用可以检测到潜在的阿尔茨海默病（AD）病理学。因此，我们根据淀粉样蛋白生物标志物的特征将两组 CBS 患者的临床、眼球运动和 18FDG-PET 成像特征进行了比较。14 例皮质基底节综合征合并淀粉样蛋白阳性（CBS-A+）的患者与 16 例皮质基底节综合征合并淀粉样蛋白阴性（CBS-A-）的患者进行了比较。两组患者的运动异常（帕金森病、肌张力障碍）和整体认知功能相似。与 CBS-A+患者表现出更多的后部皮质异常不同，CBS-A-患者在神经心理学测试、扫视速度研究和 18FDG-PET 显示的脑代谢低下区域上表现出更多的前皮质和脑干功能障碍。有趣的是，多巴胺转运蛋白 SPECT 成像显示两组患者的多巴胺能退化水平相似。这些发现证实了导致 CBS 的不同神经退行性疾病之间存在共同和不同的大脑异常。我们证明了多学科方法的重要性，特别是在眼动检查方面，可以提高体内诊断的准确性。
