What are the other Names for this Condition? (Also known as/Synonyms)

• MSA (Multiple System Atrophy)
• Shy-Drager Syndrome

What is Multiple System Atrophy? (Definition/Background Information)

• Multiple System Atrophy (MSA) is a rare and progressive neurodegenerative disorder that affects multiple systems in the body, including the autonomic nervous system and movement control. It is characterized by the progressive loss of nerve cells in specific areas of the brain. MSA is a form of dysautonomia
• The signs and symptoms of Multiple System Atrophy include autonomic dysfunction, such as orthostatic hypotension and urinary incontinence, and motor symptoms, such as tremors, muscle stiffness, and difficulties with coordination and movement. The movement-related symptoms can resemble Parkinson's disease
• A diagnosis of Multiple System Atrophy involves clinical evaluation, neuroimaging, and exclusion of other conditions. The treatment aims to manage symptoms, but the disease tends to be resistant to many therapies. The prognosis of MSA is generally poor, with progressive disability and a reduced life expectancy typically ranging from 6 to 10 years after symptom onset

Who gets Multiple System Atrophy? (Age and Sex Distribution)

• Multiple System Atrophy typically affects adults, usually between the ages of 40 and 60
• Both men and women can be affected by this condition
• The condition may occur worldwide, affecting individuals of all racial and ethnic groups

What are the Risk Factors for Multiple System Atrophy? (Predisposing Factors)

The exact risk factors for Multiple System Atrophy are not well understood.

• However, certain genetic and environmental factors may play a role
• There may also be a slightly increased risk in individuals with a family history of the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Multiple System Atrophy? (Etiology)

The exact cause of Multiple System Atrophy is presently unknown.

• However, the accumulation of an abnormal protein called alpha-synuclein in nerve cells is believed to play a role in the degeneration of these cells
• The reason for the protein accumulation and cell degeneration is not fully understood

What are the Signs and Symptoms of Multiple System Atrophy?

The signs and symptoms of Multiple System Atrophy may be mild or severe and can vary from one individual to another. These may include:

• Autonomic symptoms: Orthostatic hypotension, urinary dysfunction, constipation, and sexual dysfunction
• Movement symptoms: Parkinsonism-like symptoms, such as bradykinesia, rigidity, and postural instability
• Cerebellar symptoms: Ataxia, intention tremor, and impaired coordination
• Other symptoms: Sleep disturbances, speech difficulties, and cognitive changes (less common)

How is Multiple System Atrophy Diagnosed?

A diagnosis of Multiple System Atrophy (MSA) may involve the following tests and exams:

• Clinical evaluation: A thorough assessment of symptoms, medical history, and physical examination can help diagnose MSA
• Neurological examination: Evaluating autonomic functions, movement abnormalities, and other neurological signs
• Autonomic function tests: Assessing blood pressure regulation, heart rate response, and other autonomic functions
• Laboratory tests: These tests may be done to rule out other possible causes of symptoms
• Imaging tests, such as MRI or PET scans, may be performed to identify characteristic changes in the brain

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Multiple System Atrophy?

The complications of Multiple System Atrophy may include:

• Falls and injuries: Balance and coordination problems increase the risk of falls and injuries
• Swallowing difficulties: As the disease progresses, swallowing problems may lead to aspiration pneumonia
• Respiratory complications: Weakening of respiratory muscles can result in respiratory difficulties

How is Multiple System Atrophy Treated?

The treatment of Multiple System Atrophy may involve the following measures:

• Symptomatic management: Medications may be prescribed to alleviate specific symptoms, such as levodopa for parkinsonism symptoms, medications to manage orthostatic hypotension, and physical therapy for movement and balance issues
• Supportive care: Assisting with activities of daily living, managing swallowing difficulties, and addressing respiratory support when needed

How can Multiple System Atrophy be Prevented?

Currently, there are no known methods to prevent Multiple System Atrophy. As the cause is unclear, there are no specific preventive measures available.

What is the Prognosis of Multiple System Atrophy? (Outcomes/Resolutions)

Multiple System Atrophy (MSA) is a progressive and incurable condition.

• The prognosis varies among individuals, but the disease generally worsens over time
• The rate of progression and specific symptoms can differ, but following MSA onset, the survival period is between 6 and 10 years
• Regular medical follow-up, symptom management, and support are crucial for maintaining the best possible quality of life

Additional and Relevant Useful Information for Multiple System Atrophy:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/