Department of Neurology, University of Virginia, Charlottesville, VA, USA.
Parkinsonism Relat Disord. 2011 Mar;17(3):160-5. doi: 10.1016/j.parkreldis.2010.12.006. Epub 2010 Dec 31.
Multiple Systems Atrophy (MSA) and Dementia with Lewy bodies (DLB) can present with both REM behavior disorder and severe autonomic dysfunction. In rare occasions, patients with MSA progress to cognitive impairment and even dementia. Positron emission topography (PET) imaging using both the amyloid ligand Pittsburgh Compound B (11C-PiB) and 18 flurodeoxyglucose (18F-FDG) was used to ascertain the presence of amyloid and pattern of glucose metabolic derangement in both disorders.
Patients diagnosed with probable DLB or MSA, with clinical symptoms of either REM Behavior Disorder (RBD), Parkinsonism, or dysautonomia were prospectively identified. All underwent both 11C-PiB and 18F-FDG PET imaging. Statistical comparison between DLB, MSA, and normal controls was performed.
Six patients, 3 with DLB, 2 with Parkinson predominant MSA (MSA-P), and 1 with cerebellar predominant MSA (MSA-C) were identified. Increased level of PiB retention was noted in all patients diagnosed with DLB, but was absent in MSA. In those with DLB, glucose hypometabolism corresponded with regions of amyloid presence, and included prefrontal, parietotemporal, occipital and primary visual cortex regions. MSA patients were distinguished by cerebellar glucose hypometabolism.
These findings emphasize the distinguishing characteristics between the alpha-synuclein related disorders of DLB and MSA. The absence of amyloid in the cases of MSA is a possible distinguishing characteristic of the disorder.
多系统萎缩(MSA)和路易体痴呆(DLB)均可出现 REM 睡眠行为障碍和严重自主神经功能障碍。在极少数情况下,MSA 患者会进展为认知障碍,甚至痴呆。采用正电子发射断层扫描(PET)分别使用淀粉样蛋白配体匹兹堡复合物 B(11C-PiB)和 18 氟脱氧葡萄糖(18F-FDG)对两种疾病的淀粉样蛋白和葡萄糖代谢紊乱模式进行成像。
前瞻性地确定了被诊断为可能的 DLB 或 MSA 的患者,这些患者有 REM 行为障碍(RBD)、帕金森病或自主神经功能障碍的临床症状。所有患者均接受了 11C-PiB 和 18F-FDG PET 成像。对 DLB、MSA 和正常对照组进行了统计比较。
共发现 6 名患者,3 名患有 DLB,2 名患有帕金森为主型 MSA(MSA-P),1 名患有小脑为主型 MSA(MSA-C)。所有被诊断为 DLB 的患者均存在 PiB 保留水平升高,但 MSA 患者则没有。在 DLB 患者中,葡萄糖代谢低下与淀粉样蛋白存在区域相对应,包括额顶叶、颞顶叶、枕叶和初级视觉皮层区域。MSA 患者的特征是小脑葡萄糖代谢低下。
这些发现强调了与 DLB 和 MSA 相关的α-突触核蛋白疾病之间的区别特征。MSA 病例中缺乏淀粉样蛋白可能是该疾病的一个鉴别特征。