Terao Yasuo, Fukuda Hideki, Tokushige Shinnichi, Inomata-Terada Satomi, Yugeta Akihiro, Hamada Masashi, Ichikawa Yaeko, Hanajima Ritsuko, Ugawa Yoshikazu
Department of Neurology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.
Neurological Clinic for Children, Japan.
Clin Neurophysiol. 2016 Feb;127(2):1491-1502. doi: 10.1016/j.clinph.2015.07.035. Epub 2015 Aug 22.
Patients with multiple system atrophy (MSA) are classified into those mainly manifesting cerebellar ataxia (MSA-C) and those mainly manifesting parkinsonism (MSA-P). Pathophysiological bases of these subtypes remain unclear. We hypothesized that MSA-C patients would resemble spinocerebellar degeneration patients and MSA-P patients would resemble Parkinson's disease (PD) patients in saccade abnormalities.
We recorded visually guided and memory guided saccades (MGS) in 27 MSA-C and 15 MSA-P patients, as well as 50 age-matched normal subjects, 14 spinocerebellar degeneration patients showing pure cerebellar symptoms (SCD) and 61 Parkinson's disease (PD) patients.
Saccade parameters of both tasks showed similar changes with progressing disease in SCD and MSA-C patients, as did those of MSA-C and MSA-P patients, although hypometria was slightly more pronounced in MSA-P. In both subtypes of MSA, latency and success rate of MGS were stable throughout disease stages, whereas they deteriorated progressively with progressing disease in PD.
Pathophysiology underlying MSA-C and MSA-P is similar as viewed from saccade performance. The MGS performance in MSA was preserved. However, MSA-P patients showed more marked hypometria, suggesting a mixture of basal ganglia pathophysiology.
The similarity of saccade performance between MSA-C and MSA-P may reflect common olivopontocerebellar pathology, while the direct pathway of the basal ganglia is relatively spared compared with PD, even in MSA-P.
多系统萎缩(MSA)患者可分为主要表现为小脑性共济失调的患者(MSA-C)和主要表现为帕金森综合征的患者(MSA-P)。这些亚型的病理生理基础尚不清楚。我们推测,在扫视异常方面,MSA-C患者与脊髓小脑变性患者相似,而MSA-P患者与帕金森病(PD)患者相似。
我们记录了27例MSA-C患者、15例MSA-P患者、50名年龄匹配的正常受试者、14例表现为单纯小脑症状的脊髓小脑变性患者(SCD)和61例帕金森病(PD)患者的视觉引导扫视和记忆引导扫视(MGS)。
在SCD和MSA-C患者中,两项任务的扫视参数均随疾病进展呈现相似变化,MSA-C和MSA-P患者的扫视参数变化也相似,尽管MSA-P患者的运动减退更为明显。在MSA的两种亚型中,MGS的潜伏期和成功率在疾病各阶段均保持稳定,而在PD患者中,它们随疾病进展而逐渐恶化。
从扫视表现来看,MSA-C和MSA-P的病理生理学相似。MSA患者的MGS表现得以保留。然而,MSA-P患者表现出更明显的运动减退,提示存在基底神经节病理生理学的混合情况。
MSA-C和MSA-P之间扫视表现的相似性可能反映了常见的橄榄脑桥小脑病变,而与PD相比,即使在MSA-P中,基底神经节的直接通路相对未受影响。