Tokuhara Yusuke, Watanabe Shohei, Yoshikawa Hiroo
Department of Internal Medicine, Division of Neurology, Hyogo College of Medicine, Nishinomiya 663-8501, Japan.
Department of Neurology, Nippon Life Hospital, Osaka 550-0006, Japan.
Clin Park Relat Disord. 2020 Apr 22;3:100054. doi: 10.1016/j.prdoa.2020.100054. eCollection 2020.
Multiple system atrophy (MSA) is an adult-onset progressive neurodegenerative disease that causes parkinsonism, cerebellar ataxia, and/or autonomic failure. MSA is categorized as MSA with predominant cerebellar ataxia (MSA-C) or MSA with predominant parkinsonism (MSA-P) according to the cardinal symptom at diagnosis. MSA-C has been reported to be the predominant presentation in Japan to date. However, major epidemiological studies regarding MSA in Japan were carried out before 2006; thus, the recent advancement of various imaging studies remains unclear. This study aimed to investigate the clinical characteristics of the recent MSA patients in Japan.
In this retrospective study, we divided 80 probable MSA patients into group A and group B and examined them to reveal whether the clinical features of MSA were different depending on the time periods of diagnosis (1989-2003 and 2004-2018, respectively).
The age at onset was significantly higher in MSA-P patients than in MSA-C patients ( = 0.0039) and was also higher in group B than in group A ( = 0.013). Although MSA-C was the predominant type in both groups, MSA-P was significantly more frequent in group B than in group A ( = 0.039). Although not statistically significant, the early heart/mediastinum ratio in [I]-meta-iodo benzylguanidine (MIBG) myocardial scintigraphy tended to be lower in patients with MSA-P than in those with MSA-C.
The proportion of MSA-P was likely larger than previously recorded due to the aging population in Japan and the improvement of differential diagnosis between PD and MSA-P.
多系统萎缩（MSA）是一种成人起病的进行性神经退行性疾病，可导致帕金森综合征、小脑共济失调和/或自主神经功能衰竭。根据诊断时的主要症状，MSA可分为以小脑共济失调为主的MSA（MSA-C）或以帕金森综合征为主的MSA（MSA-P）。据报道，迄今为止MSA-C是日本最主要的表现形式。然而，日本关于MSA的主要流行病学研究是在2006年之前进行的；因此，各种影像学研究的最新进展仍不清楚。本研究旨在调查日本近期MSA患者的临床特征。
在这项回顾性研究中，我们将80例可能的MSA患者分为A组和B组，并对他们进行检查，以揭示MSA的临床特征是否因诊断时间段（分别为1989 - 2003年和2004 - 2018年）而有所不同。
MSA-P患者的发病年龄显著高于MSA-C患者（ = 0.0039），B组患者的发病年龄也高于A组（ = 0.013）。虽然MSA-C在两组中都是主要类型，但MSA-P在B组中的发生率显著高于A组（ = 0.039）。虽然无统计学意义，但在[I]-间碘苄胍（MIBG）心肌闪烁显像中，MSA-P患者的早期心脏/纵隔比值往往低于MSA-C患者。
由于日本人口老龄化以及帕金森病（PD）和MSA-P之间鉴别诊断的改善，MSA-P的比例可能比以前记录的更大。
