From the Guidelines
Multiple System Atrophy (MSA) is a rare, progressive neurodegenerative disorder characterized by a combination of parkinsonian, cerebellar, and autonomic symptoms, with a mean disease duration of almost 6 years, as reported in the 2020 study by the Journal of the American College of Radiology 1. MSA typically affects adults in their 50s and 60s, with a life expectancy of about 7-10 years after diagnosis. The disorder is divided into three distinct clinical subtypes: MSA-P (striatonigral degeneration), MSA-C (olivopontocerebellar atrophy), and MSA-A (Shy-Drager syndrome), each with predominant features of parkinsonism, cerebellar ataxia, or autonomic dysfunction, respectively 1. Some key characteristics of MSA include:
- Severe autonomic dysfunction, such as orthostatic hypotension, urinary incontinence, and constipation
- Poor response to levodopa therapy
- Rapid progression of symptoms
- Specific brain changes visible on MRI, such as the "hot cross bun" sign in the pons and putaminal atrophy Treatment of MSA is primarily symptomatic, focusing on managing orthostatic hypotension, addressing urinary symptoms, and maintaining mobility through physical therapy 1. The current approach to managing MSA prioritizes comprehensive supportive care to maintain quality of life, as no disease-modifying treatments are currently available 1.
From the Research
Definition and Characteristics of MSA
- Multiple system atrophy (MSA) is a rare, progressive, and fatal neurodegenerative disorder 2, 3, 4, 5, 6.
- It is characterized by a combination of autonomic failure, poorly levodopa-responsive parkinsonism, and cerebellar ataxia 2, 4, 5, 6.
- The disease can be divided into two main types: the parkinsonian type (MSA-P) and the cerebellar type (MSA-C) 3, 5.
Clinical Features of MSA
- The clinical picture of MSA includes genitourinary dysfunction, orthostatic hypotension, and rapid eye movement (REM) sleep behavior disorder 3, 5.
- Patients with MSA-C rapidly develop balance, speech, and coordination abnormalities, with ataxia being a prominent feature 3.
- Autonomic dysfunction, parkinsonism, and REM sleep disorder are also common features of MSA 3, 5.
Treatment and Management of MSA
- The treatment of MSA is mainly symptomatic, with a focus on managing autonomic failure, alleviating parkinsonism and cerebellar ataxia, and associated disabilities 2, 3, 6.
- Current therapeutic strategies are primarily based on dopamine replacement and improvement of autonomic failure, but these have limited effectiveness 2.
- There are ongoing efforts to develop disease-modifying agents, and progress is being made towards early diagnosis and the development of sensitive diagnostic and progression biomarkers for MSA 6.