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Multiple System Atrophy - State of the Art.
Brice Laurens1, Sylvain Vergnet1, Miguel Cuina Lopez2,3
1Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux, 33000, Bordeaux, France.
Current Neurology and Neuroscience Reports
|April 6, 2017
Summary
Multiple system atrophy (MSA) is a rare neurodegenerative disease. This review critically examines recent research on MSA pathogenesis, genetics, diagnostics, and treatments, highlighting unmet needs in neuroprotection.
Area of Science:
- Neuroscience
- Neuropathology
- Genetics
Background:
- Multiple system atrophy (MSA) is a rare, fatal neurodegenerative disorder.
- Characterized by parkinsonism, cerebellar, and autonomic dysfunction.
- Pathologic hallmark: alpha-synuclein (α-syn) aggregates in oligodendrocytes (glial cytoplasmic inclusions).
Purpose of the Study:
- Critically review recent studies (past 5 years) on MSA.
- Focus on pathogenesis, genetics, clinical signs, biomarkers, and treatment development.
- Address the ongoing debate on the origin of α-syn aggregates in oligodendrocytes.
Main Methods:
- Literature review of studies published in the last 5 years.
- Critical analysis of research on MSA pathogenesis.
- Synthesis of findings related to genetics, clinical presentation, and biomarkers.
Main Results:
- Identified unmet needs in neuroprotection and disease-modification for MSA.
- Highlighted the role of alpha-synuclein aggregation as a key pathological feature.
- Summarized progress and ongoing debates in understanding MSA origins.
Conclusions:
- Despite progress, the origin of alpha-synuclein aggregates in MSA oligodendrocytes remains debated.
- Current treatments are symptomatic; disease-modifying therapies are needed.
- Further research into pathogenesis, biomarkers, and genetics is crucial for developing effective treatments.