Current Landscape of Clinical Diagnosis in Multiple System Atrophy
Hiroaki Sekiya, Philip W. Tipton, Miki Kawazoe, Shunsuke Koga, Aya Murakami, Alexia R. Maier, Ryan J. Uitti, William P. Cheshire, Zbigniew K. Wszołek, Dennis W. Dickson
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: = 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.