From the Guidelines
Multiple System Atrophy (MSA) is a rare, progressive neurodegenerative disorder characterized by the accumulation of alpha-synuclein in brain cells, leading to symptoms similar to Parkinson's disease, as well as autonomic dysfunction, with a typical onset between 55 to 65 years of age and a mean disease duration of almost 6 years 1.
Key Characteristics of MSA
- Affects multiple systems in the body, including the autonomic nervous system
- Causes symptoms such as slow movement, rigid muscles, and poor balance
- Involves the accumulation of alpha-synuclein in certain brain cells, particularly those that produce dopamine
- Can be subdivided into three distinct clinical subtypes: MSA-P (striatonigral degeneration), MSA-C (olivopontocerebellar atrophy), and MSA-A (Shy-Drager syndrome)
Diagnosis and Treatment
- Correctly diagnosing MSA on clinical features alone can be challenging, and imaging remains an essential diagnostic tool 1
- Treatment focuses on managing symptoms with medications, physical therapy, occupational therapy, and speech therapy to maintain function and quality of life
- There is currently no cure for MSA, and it is ultimately fatal, with respiratory complications being a common cause of death
Important Considerations
- Early diagnosis is important for appropriate symptom management and planning
- MSA can be difficult to distinguish from Parkinson's disease in early stages, highlighting the need for careful evaluation and diagnosis 1
From the Research
Definition and Characteristics of Multiple System Atrophy (MSA)
- Multiple System Atrophy (MSA) is a progressive neurodegenerative disease characterized by motor, nonmotor, and autonomic symptoms 2, 3, 4.
- It results from putaminal and cerebellar degeneration and is associated with glial cytoplasmic inclusions enriched with α-synuclein in oligodendrocytes and neurons 2.
- MSA is a rare neurodegenerative disorder without any effective treatment in slowing or stopping disease progression 3.
Clinical Features of MSA
- MSA presents with autonomic failure and a complicated motor syndrome including atypical parkinsonism, ataxia, and pyramidal signs 4.
- It affects adults and is characterized by autonomic failure affecting many systems, including cardiovascular, urinary, sexual, gastrointestinal, and sudomotor systems 5.
- The disease is also associated with poor levodopa-responsive Parkinsonism, cerebellar ataxia, pyramidal signs, and autonomic failure in any combination 3.
Disease Progression and Prognostic Factors
- MSA has a rapid progression and poor prognosis, with a median survival of 4.0 years since the first visit 6.
- The main factors for poor survival include the progression over time of UMSARS score and the severity of orthostatic hypotension 6.
- Early autonomic dysfunction and the severity of orthostatic hypotension have a negative impact on both disease progression and survival 6.
Management and Treatment of MSA
- Current therapeutic strategies are primarily based on dopamine replacement and improvement of autonomic failure 3.
- Symptomatic management remains disappointing, and no curative treatment is yet available 3.
- Research into disease-modifying therapies is ongoing, with a particular focus on synuclein-targeted therapies among others 2.