Multiple system atrophy.

Fanciulli, Alessandra; Krismer, Florian; Levin, Johannes; Stankovic, Iva; Wenning, Gregor K; Seppi, Klaus

doi:10.1016/bs.irn.2019.10.004

Journal Article

DZNE-2020-00491

Multiple system atrophy.

Fanciulli, A. (Corresponding author) ; Stankovic, I. ; Krismer, F. ; Seppi, K. ; Levin, J.DZNE* ; Wenning, G. K.

2019
Elsevier New York, NY [u.a.]

International review of neurobiology 149, 137-192 (2019) [10.1016/bs.irn.2019.10.004]

This record in other databases:

Please use a persistent id in citations: doi:10.1016/bs.irn.2019.10.004

Abstract: Multiple system atrophy (MSA) is a sporadic, adult-onset, relentlessly progressive neurodegenerative disorder, clinically characterized by various combinations of autonomic failure, parkinsonism and ataxia. The neuropathological hallmark of MSA are glial cytoplasmic inclusions consisting of misfolded α-synuclein. Selective atrophy and neuronal loss in striatonigral and olivopontocerebellar systems underlie the division into two main motor phenotypes of MSA-parkinsonian type and MSA-cerebellar type. Isolated autonomic failure and REM sleep behavior disorder are common premotor features of MSA. Beyond the core clinical symptoms, MSA manifests with a number of non-motor and motor features. Red flags highly specific for MSA may provide clues for a correct diagnosis, but in general the diagnostic accuracy of the second consensus criteria is suboptimal, particularly in early disease stages. In this chapter, the authors discuss the historical milestones, etiopathogenesis, neuropathological findings, clinical features, red flags, differential diagnosis, diagnostic criteria, imaging and other biomarkers, current treatment, unmet needs and future treatments for MSA.

Keyword(s): Humans (MeSH) ; Multiple System Atrophy: diagnosis (MeSH) ; Multiple System Atrophy: pathology (MeSH) ; Multiple System Atrophy: physiopathology (MeSH) ; Multiple System Atrophy: therapy (MeSH)