Dataset Information

Laryngeal Movement Disorders in Multiple System Atrophy: A Diagnostic Biomarker?

ABSTRACT:

Background

Multiple system atrophy (MSA) is a rare neurodegenerative disorder, and its parkinsonian variant can be difficult to delineate from Parkinson's disease (PD). Despite laryngeal dysfunction being associated with decreased life expectancy and quality of life, systematic assessments of laryngeal dysfunction in large cohorts are missing.

Objectives

The objective of this study was to systematically assess laryngeal dysfunction in MSA and PD and identify laryngeal symptoms that allow for differentiating MSA from PD.

Methods

Patients with probable or possible MSA underwent flexible endoscopic evaluation of swallowing performing a systematic task protocol. Findings were compared with an age-matched PD cohort.

Results

A total of 57 patients with MSA (64 [59-71] years; 35 women) were included, and task assessments during endoscopic examination compared with 57 patients with PD (67 [60-73]; 28 women). Patients with MSA had a shorter disease duration (4 [3-5] years vs 7 [5-10]; P?ConclusionLaryngeal movement disorders are highly prevalent in patients with MSA when assessed by a specific task protocol despite the lack of overt clinical symptoms. Our data suggest that irregular arytenoid cartilage movements could be used as a clinical marker to delineate MSA from PD with a specificity of 1.0 and sensitivity 0.9. © 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

SUBMITTER: Gandor F

PROVIDER: S-EPMC7818263 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature

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Publications

Laryngeal Movement Disorders in Multiple System Atrophy: A Diagnostic Biomarker?

Gandor Florin F Vogel Annemarie A Claus Inga I Ahring Sigrid S Gruber Doreen D Heinze Hans-Jochen HJ Dziewas Rainer R Ebersbach Georg G Warnecke Tobias T

Movement disorders : official journal of the Movement Disorder Society 20200805 12

<h4>Background</h4>Multiple system atrophy (MSA) is a rare neurodegenerative disorder, and its parkinsonian variant can be difficult to delineate from Parkinson's disease (PD). Despite laryngeal dysfunction being associated with decreased life expectancy and quality of life, systematic assessments of laryngeal dysfunction in large cohorts are missing.<h4>Objectives</h4>The objective of this study was to systematically assess laryngeal dysfunction in MSA and PD and identify laryngeal symptoms tha ...[more]

PMID: 32757231

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Project description:Multiple system atrophy (MSA) is an orphan, fatal, adult-onset neurodegenerative disorder of uncertain etiology that is clinically characterized by various combinations of parkinsonism, cerebellar, autonomic, and motor dysfunction. MSA is an ?-synucleinopathy with specific glioneuronal degeneration involving striatonigral, olivopontocerebellar, and autonomic nervous systems but also other parts of the central and peripheral nervous systems. The major clinical variants correlate with the morphologic phenotypes of striatonigral degeneration (MSA-P) and olivopontocerebellar atrophy (MSA-C). While our knowledge of the molecular pathogenesis of this devastating disease is still incomplete, updated consensus criteria and combined fluid and imaging biomarkers have increased its diagnostic accuracy. The neuropathologic hallmark of this unique proteinopathy is the deposition of aberrant ?-synuclein in both glia (mainly oligodendroglia) and neurons forming glial and neuronal cytoplasmic inclusions that cause cell dysfunction and demise. In addition, there is widespread demyelination, the pathogenesis of which is not fully understood. The pathogenesis of MSA is characterized by propagation of misfolded ?-synuclein from neurons to oligodendroglia and cell-to-cell spreading in a "prion-like" manner, oxidative stress, proteasomal and mitochondrial dysfunction, dysregulation of myelin lipids, decreased neurotrophic factors, neuroinflammation, and energy failure. The combination of these mechanisms finally results in a system-specific pattern of neurodegeneration and a multisystem involvement that are specific for MSA. Despite several pharmacological approaches in MSA models, addressing these pathogenic mechanisms, no effective neuroprotective nor disease-modifying therapeutic strategies are currently available. Multidisciplinary research to elucidate the genetic and molecular background of the deleterious cycle of noxious processes, to develop reliable biomarkers and targets for effective treatment of this hitherto incurable disorder is urgently needed.

Dataset Information

Laryngeal Movement Disorders in Multiple System Atrophy: A Diagnostic Biomarker?

Background

Objectives

Methods

Results

Publications

Laryngeal Movement Disorders in Multiple System Atrophy: A Diagnostic Biomarker?

Similar Datasets

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