From the Departments of Neuroscience, Rehabilitation, Ophthalmology, Genetics, and Maternal and Child Health (M.P., F.N.) and Health Sciences (S.M.), University of Genoa; IRCCS Ospedale Policlinico San Martino (M.P., S.M., F.N.), Genoa, Italy; Cognitive Neuroscience Division, Department of Neurology (E.D.H.), Gertrude H. Sergievsky Center, New York; Taub Institute for Research on Alzheimer's Disease and the Aging Brain (E.D.H., W.C.K.), Columbia University Medical Center, New York, NY; Department of Neurology (S.S.), UCSF Memory and Aging Center, UCSF, San Francisco, CA; Department of Pathology and Laboratory Medicine (S.S., B.G.), Indiana University School of Medicine, Indianapolis; Nuclear Medicine Unit (S.M.), IRCCS AOU San Martino, IST, Genoa, Italy; Behavioral Neurology Unit (E.M.W.), National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD; Psychiatry and Behavioral Sciences & Cognitive Neurology/Alzheimer's Disease Research Center (J.G.), Feinberg School of Medicine and Department of Psychology, Northwestern University; and Brain Injury Research, Cognitive Neuroscience Lab, Think and Speak Lab (J.G.), Shirley Ryan AbilityLab, Chicago, IL.
Neurology. 2019 Mar 5;92(10):e1121-e1135. doi: 10.1212/WNL.0000000000007038. Epub 2019 Jan 30.
To evaluate brain Fluorodeoxyglucose PET (FDG-PET) differences among patients with a clinical diagnosis of corticobasal syndrome (CBS) and distinct underling primary pathologies.
We studied 29 patients with a diagnosis of CBS who underwent FDG-PET scan and postmortem neuropathologic examination. Patients were divided into subgroups on the basis of primary pathologic diagnosis: CBS-corticobasal degeneration (CBS-CBD) (14 patients), CBS-Alzheimer disease (CBS-AD) (10 patients), and CBS-progressive supranuclear palsy (CBS-PSP) (5 patients). Thirteen age-matched healthy patients who underwent FDG-PET were the control group (HC). FDG-PET scans were compared between the subgroups and the HC using SPM-12, with a threshold of < 0.05.
There were no differences in Mattis Dementia Rating Scale or finger tapping scores between CBS groups. Compared to HC, the patients with CBS presented significant hypometabolism in frontoparietal regions, including the perirolandic area, basal ganglia, and thalamus of the clinically more affected hemisphere. Patients with CBS-CBD showed a similar pattern with a more marked, bilateral involvement of the basal ganglia. Patients with CBS-AD presented with posterior, asymmetric hypometabolism, including the lateral parietal and temporal lobes and the posterior cingulate. Finally, patients with CBS-PSP disclosed a more anterior hypometabolic pattern, including the medial frontal regions and the anterior cingulate. A conjunction analysis revealed that the primary motor cortex was the only common area of hypometabolism in all groups, irrespective of pathologic diagnosis.
In patients with CBS, different underling pathologies are associated with different patterns of hypometabolism. Our data suggest that FDG-PET scans could help in the etiologic diagnosis of CBS.
评估临床诊断为皮质基底节综合征(CBS)且具有不同潜在主要病理学的患者之间的脑氟脱氧葡萄糖正电子发射断层扫描(FDG-PET)差异。
我们研究了 29 名接受 FDG-PET 扫描和死后神经病理学检查的临床诊断为 CBS 的患者。根据主要病理诊断将患者分为亚组:CBS-皮质基底节变性(CBS-CBD)(14 名患者)、CBS-阿尔茨海默病(CBS-AD)(10 名患者)和 CBS-进行性核上性麻痹(CBS-PSP)(5 名患者)。13 名年龄匹配的接受 FDG-PET 的健康患者为对照组(HC)。使用 SPM-12 对亚组和 HC 之间的 FDG-PET 扫描进行比较,阈值为 < 0.05。
CBS 组之间的 Mattis 痴呆评定量表或指敲击评分无差异。与 HC 相比,CBS 患者的额顶区域,包括优势半球的旁中央区、基底节和丘脑,存在明显的代谢低下。CBS-CBD 患者表现出相似的模式,基底节的双侧受累更为明显。CBS-AD 患者表现为后不对称代谢低下,包括外侧顶叶和颞叶以及后扣带回。最后,CBS-PSP 患者表现出更靠前的代谢低下模式,包括内侧额叶和前扣带回。联合分析显示,原发性运动皮层是所有组中唯一共同的代谢低下区域,与病理诊断无关。
在 CBS 患者中,不同的潜在病理学与不同的代谢低下模式相关。我们的数据表明,FDG-PET 扫描可能有助于 CBS 的病因诊断。