Multiple System Atrophy
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Definition/Description
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Multiple System Atrophy (MSA) is defined as a sporadic, progressive, neurodegenerative adult-onset disorder that can affect the autonomic system, basal ganglia causing parkinsonism, and/or cerebellum causing ataxia in any combination.(Lundy-Eckman)[1]
This disorder is called Multiple System Atrophy, because there is a combination of symptoms arising from each system just mentioned (autonomic system, basal gangila, and cerebellum). In order for a patient to be diagnosed with MSA, there have to be at least two systems involved. There can be any combination of symptoms present from the three systems. (Swan)[2]
- In MSA, patients often have symptoms that are characteristic of autonomic dysfunction, parkinsonism, or cerebellar dysfunction. As the disease progresses, additional symptoms emerge (Swan).[2]
- Some patients may initially have autonomic features and may be diagnosed with pure autonomic failure. (Swan)[2]
o Patients who are initially misdiagnosed with PAF may later be correctly diagnosed with MSA as the neurological symptoms of parkinsonism and/or cerebellar ataxia appear. (Swan).[2]
There are three different categories or types of MSA, based upon what areas of the nervous system or autonomic system are affected and to what degree:
- Clinically, MSA is dominated by autonomic failure (MSA-A subtype) (also referred to as Shy Drager Syndrome), which may be associated with either:
- the movement dysfunction of parkinsonism (MSA-P subtype) (also referred to as Striatonigral degeneration) in 80% of cases. 2,3[3][4]
- or with cerebellar ataxia (MSA-C subtype) (also referred to as Sporadic Olivopontocerebellar atrophy OPCA), which will result in difficulty with postural control and coordination in 20-50% of cases. 2,3,[3][4]
Prevalence[edit | edit source]
The prevalence of MSA has been estimated to be 4.4 per 100,000 people. (Diedrich).[5]
The mean age at onset has been reported to be between 52.5 and 55 years (Diedrich).[5]
MSA is found more often in men than in women (Diedrich).[5]
Wenning et al- completed an analysis of 100 patients with MSA and measured their disability. They found that patients with MSA become disabled at a faster rate than patients with Parkinson's Disease.[3]
Characteristics/Clinical Presentation[edit | edit source]
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Systemic Involvement[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
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Physical Therapy Management (current best evidence)[edit | edit source]
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References[edit | edit source]
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- ↑ Lundy-Eckman, L. Neuroscience: Fundamentals for Rehabilitation. 3rd ed. St. Louis: Saunders Elsevier, 2002.
- ↑ 2.0 2.1 2.2 2.3 Swan L, Dupont J. Multiple System Atrophy. Journal of Physical Therapy 1999;79:488-94.
- ↑ 3.0 3.1 3.2 Wenning GK, Braune S. Multiple System Atrophy: Pathophysiology and Management. CNS Drugs 2001;15:839-48.
- ↑ 4.0 4.1 Wedge F. The Impact of Resistance Training on Balance and Functional Ability of a Patient with Multiple System Atrophy. Journal of Geriatric Physical Therapy 2008;31:79-83.
- ↑ 5.0 5.1 5.2 Diedrich A, Robertson D. Multiple System Atrophy. Vanderbilt University School of Medicine 2009. http://emedicine.medscape.com/article/1154583-overview (accessed on 24 Jan 2010).