Current Landscape of Clinical Diagnosis in Multiple System Atrophy: A 15-Year Analysis From 2008 to 2022.

IF 7.7 1区医学 Q1 CLINICAL NEUROLOGY Neurology Pub Date : 2024-12-10 Epub Date: 2024-11-12 DOI:10.1212/WNL.0000000000210021

Hiroaki Sekiya, Philip W Tipton, Miki Kawazoe, Shunsuke Koga, Aya Murakami, Alexia R Maier, Ryan J Uitti, William P Cheshire, Zbigniew K Wszolek, Dennis W Dickson

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Abstract

Background and objectives: Clinical diagnosis of multiple system atrophy (MSA) is challenging. In 2022, new diagnostic criteria for MSA were proposed. We hypothesized that the positive predictive value (PPV) of clinical diagnosis of MSA improved because of advanced diagnostic tools, including brain MRI. This study aimed to understand temporal changes in PPV of MSA.

Methods: We conducted a retrospective analysis of patients clinically diagnosed with MSA whose brains were examined in the Mayo Clinic brain bank from 2008 to 2022. PPV was compared between 2 periods (2008-2017 and 2018-2022) and successively with a 4-year moving average. PPV for each clinical subtype (parkinsonism type [MSA-P] and cerebellar type [MSA-C]) was assessed.

Results: This study included 321 patients (136 women, age at death 68 ± 9 years) with a clinical diagnosis of MSA. Among them, 225 were pathologically confirmed as MSA, resulting in an overall PPV of 70%. The remaining 30% had alternative pathologic diagnoses including Lewy body disease (18%), progressive supranuclear palsy (4%), cerebrovascular disease (1%), corticobasal degeneration (1%), and others (6%). PPV improved from 63% in 2008-2017 to 78% in 2018-2022 (odds ratio [OR] 2.0 [1.2-3.5], p = 0.005). Linear analysis also demonstrated increased PPV over time (r = 0.66 [0.14-0.89], p = 0.02). Brain MRI scans were more frequently performed in 2018-2022 compared with 2008-2017 (91% vs 80%; OR 2.4 [1.2-5.0], p = 0.012). PPV was higher in patients with brain MRI compared with those without (73% vs 52%; OR 2.5 [1.3-4.9], p = 0.0057). PPV for MSA-C was similar in both groups (87% in 2008-2017 and 93% in 2018-2022), while that for MSA-P improved from 59% in 2008-2017 to 72% in 2018-2022 (OR 1.8 [1.0-3.2], p = 0.04).

Discussion: This study demonstrates an improvement in the PPV of MSA in recent years, potentially attributed to the increased use of brain MRI. Nevertheless, it also highlights that it remains difficult to make a correct diagnosis for some patients based on their clinical presentation. These findings provide a baseline for future clinicopathologic research on MSA.

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多系统萎缩症临床诊断的现状：从 2008 年到 2022 年的 15 年分析。

背景和目的：多系统萎缩（MSA）的临床诊断具有挑战性。2022 年，提出了 MSA 的新诊断标准。我们假设，由于包括脑磁共振成像在内的先进诊断工具的出现，MSA临床诊断的阳性预测值（PPV）有所提高。本研究旨在了解 MSA PPV 的时间变化：我们对临床诊断为MSA的患者进行了回顾性分析，这些患者的大脑在2008年至2022年期间接受了梅奥诊所脑库的检查。PPV在两个时期（2008-2017年和2018-2022年）之间进行比较，并连续使用4年移动平均值。评估了各临床亚型（帕金森病型[MSA-P]和小脑型[MSA-C]）的PPV：本研究纳入了 321 名临床诊断为 MSA 的患者（136 名女性，死亡时年龄为 68 ± 9 岁）。其中 225 人经病理证实为 MSA，总 PPV 为 70%。其余30%的患者有其他病理诊断，包括路易体病（18%）、进行性核上性麻痹（4%）、脑血管病（1%）、皮质基底变性（1%）和其他（6%）。PPV从2008-2017年的63%提高到2018-2022年的78%（比值比 [OR] 2.0 [1.2-3.5]，P = 0.005）。线性分析也显示 PPV 随时间推移而增加（r = 0.66 [0.14-0.89]，p = 0.02）。与 2008-2017 年相比，2018-2022 年进行脑磁共振扫描的频率更高（91% vs 80%；OR 2.4 [1.2-5.0]，p = 0.012）。与未进行脑磁共振成像的患者相比，进行脑磁共振成像的患者的 PPV 更高（73% vs 52%；OR 2.5 [1.3-4.9]，p = 0.0057）。两组MSA-C的PPV相似（2008-2017年为87%，2018-2022年为93%），而MSA-P的PPV从2008-2017年的59%提高到2018-2022年的72%（OR 1.8 [1.0-3.2]，p = 0.04）：本研究表明，近年来 MSA 的 PPV 有所提高，这可能归功于脑磁共振成像的使用增加。尽管如此，该研究也强调了根据临床表现对某些患者做出正确诊断仍然存在困难。这些发现为未来的MSA临床病理学研究提供了一个基线。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neurology 医学-临床神经学

CiteScore

12.20

自引率

4.00%

发文量

1973

审稿时长

2-3 weeks

期刊介绍： Neurology, the official journal of the American Academy of Neurology, aspires to be the premier peer-reviewed journal for clinical neurology research. Its mission is to publish exceptional peer-reviewed original research articles, editorials, and reviews to improve patient care, education, clinical research, and professionalism in neurology. As the leading clinical neurology journal worldwide, Neurology targets physicians specializing in nervous system diseases and conditions. It aims to advance the field by presenting new basic and clinical research that influences neurological practice. The journal is a leading source of cutting-edge, peer-reviewed information for the neurology community worldwide. Editorial content includes Research, Clinical/Scientific Notes, Views, Historical Neurology, NeuroImages, Humanities, Letters, and position papers from the American Academy of Neurology. The online version is considered the definitive version, encompassing all available content. Neurology is indexed in prestigious databases such as MEDLINE/PubMed, Embase, Scopus, Biological Abstracts®, PsycINFO®, Current Contents®, Web of Science®, CrossRef, and Google Scholar.