Multisystematrophie

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Authors: Ida Jensen, Sabrina Katzdobler, Alexander Bernhardt, Günter Höglinger, Franziska Hopfner
Cited by: 1
References: 43

Abstract

Abstract: Multiple system atrophy is a rare, rapidly progressive neurodegenerative disorder within the spectrum of atypical parkinsonian syndromes. It is clinically defined by the combination of parkinsonism, cerebellar ataxia, and autonomic failure. Neuropathological findings reveal misfolded α-synuclein accumulation in oligodendrocytes, leading to multisystem neurodegeneration. Diagnosis is based on the Movement Disorder Society criteria and characteristic MRI findings but can only be definitively confirmed post mortem. Currently, no disease-modifying therapies are available; management is symptomatic and requires a multidisciplinary approach. Ongoing research aims to establish sensitive biomarkers, identify prodromal disease stages, and develop targeted interventions addressing key pathophysiological mechanisms such as α-synuclein aggregation and neuroinflammation.

Fields of Study & Subjects

Parkinson's Disease Mechanisms and Treatments Neurological diseases and metabolism Neurological disorders and treatments

Publication details

Journal / source
Fortschritte der Neurologie · Psychiatrie
Publisher
Georg Thieme Verlag KG
Volume
94
Issue
09
Pages
377–388
Publication date
6 August 2026
Language
DE