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Multiple-system atrophy, a rare adult-onset neurodegenerative disease, is associated with autonomic dysfunction, features of parkinsonism, and cerebellar ataxia. This article discusses multiple-system atrophy pathophysiology, clinical presentation, and perioperative issues pertinent to anesthesia.
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http://dx.doi.org/10.1213/XAA.0000000000000939 | DOI Listing |
Neurology
October 2025
Montreal Neurological Institute-Hospital, McGill University, Montreal, Canada.
Background And Objectives: Years before diagnosis of Parkinson disease (PD), dementia with Lewy bodies (DLB), or multiple system atrophy (MSA), mild prodromal manifestations can be detected. Longitudinal follow-up of people with prodromal synucleinopathy, particularly idiopathic/isolated REM sleep behavior disorder (iRBD), enables in-depth clinical phenotyping of early disease, which could facilitate stratification for clinical trials, provide the definition of appropriate end points, or predict phenoconversion more precisely. The aim of this study was to update and expand on previous studies assessing clinical evolution from iRBD to clinically diagnosed disease, up to 14 years before diagnosis.
View Article and Find Full Text PDFBrain
September 2025
IRCCS Istituto delle Scienze Neurologiche di Bologna, UOC Clinica Neurologica, Bologna, 40139, Italy.
An early diagnosis of Parkinson's disease (PD) represents a challenge and novel accurate biomarkers are therefore urgently needed. Detection of phosphorylated α-synuclein (p-α-syn) in skin nerve fibers has shown promise as such a marker. However, its accuracy for the identification of PD among patients with early signs of parkinsonism has not been thoroughly explored.
View Article and Find Full Text PDFNeurodegener Dis Manag
September 2025
RWE Statistics, KMK Consulting, Inc, North Tower, Morristown, NJ, USA.
Background: Multiple system atrophy (MSA) is a progressive neurodegenerative disorder with diverse symptoms that complicate diagnosis. We aimed to characterize MSA-related symptoms, medications, and healthcare resource utilization (HCRU).
Research Design And Methods: This retrospective cohort study used a large US claims database.
Cerebellum
September 2025
Department of Neurology, Faculty of Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Multiple system atrophy (MSA) is a progressive, adult-onset neurodegenerative disorder involving autonomic failure, cerebellar ataxia, and parkinsonism. Patients often require invasive interventions, such as gastrostomy or tracheostomy, and sudden death is common. This study aimed to elucidate patterns of invasive treatment and identify risk factors for tracheostomy or sudden death within 5 years of onset.
View Article and Find Full Text PDFJ Neurol
September 2025
Department of Neurology, Seoul National University Hospital and Seoul National University College of Medicine, 101 Daehakro Jongno-gu, Seoul, 03080, Republic of Korea.
Speech disorders differ between Parkinson's disease (PD) and multiple system atrophy (MSA), but studies focusing on group differences based on syllables or including cerebellar ataxia (CA) are lacking until now. This cross-sectional study aimed to analyze syllable-based speech characteristics in patients with PD, MSA, and CA, as well as healthy controls, to determine their diagnostic utility. Speech samples were collected from 68 PD, 52 MSA, 23 CA, and 70 healthy controls.
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