Multiple system atrophy (MSA)

Multiple system atrophy (MSA), also known as Shy-Drager Syndrome, is a complex neurological disorder that presents with a range of motor and autonomic symptoms due to its effect on the autonomic nervous system.

While there is currently no cure for multiple system atrophy, early diagnosis and appropriate management can help alleviate symptoms and improve the quality of life for affected individuals.

If you or someone you know experience any of the symptoms associated with multiple system atrophy, it’s crucial to seek medical attention promptly.

What is multiple system atrophy?

Multiple system atrophy is a rare and progressive neurological disorder that affects various systems within the body. Think of it like a glitch in the body's operating system, causing disruptions in movement, balance and involuntary functions like blood pressure and bladder control.

Unlike other neurodegenerative diseases such as Parkinson's disease or multiple sclerosis (MS), multiple system atrophy doesn't just focus on one area of the brain or nervous system. Instead, it's like a multitasking troublemaker, impacting multiple systems simultaneously.

For example, Parkinson's disease primarily affects movement and is characterized by tremors, stiffness and slowness of movement. On the other hand, multiple sclerosis mainly targets the central nervous system, causing damage to the protective covering of nerve fibers (myelin sheath), leading to a wide range of symptoms including fatigue, muscle weakness and vision problems.

Types of multiple system atrophy

MSA comes in two main types: MSA-P and MSA-C. Think of them as two different versions of the same problem.

• MSA-P: Primarily affects movement (the "P" stands for Parkinsonism), leading to symptoms similar to Parkinson's disease, such as muscle stiffness, tremors and difficulty with coordination and balance.
• MSA-C: Primarily affects cerebellar functions (the "C" stands for cerebellar), leading to problems with coordination, balance and speech. This type may not have as many movement-related symptoms initially, but it can still cause significant difficulties in daily life.

What are the symptoms of MSA?

Multiple system atrophy can bring about various symptoms that impact movement and bodily functions. It's like dealing with a bunch of glitches in the body's control systems, causing troubles in different areas all at once.

Multiple system atrophy symptoms may include:

• Low blood pressure
• Dizziness, feeling lightheaded or fainting
• Urinary and bowel dysfunction
• Constipation
• Loss of bladder or bowel control (incontinence)
• Changes in sweat production
• Heat intolerance due to reduced sweating
• Impaired body temperature control, often causing cold hands or feet
• Sleep disorders
• Impotence in men
• Loss of libido
• Color changes in hands and feet caused by pooling of blood
• Difficulty controlling emotions, such as laughing or crying inappropriately

While these are general symptoms, each type of multiple system atrophy presents its own set of symptoms.

MSA-P symptoms

• Muscle stiffness
• Slow movement
• Tremors in your arms and legs
• Difficulty bending your arms and legs
• Balance and posture problems

MSA-C symptoms

• Loss of muscle coordination (ataxia)
• Impaired ability to walk and keep your balance
• Slurred speech or soft speaking
• Blurred or double vision
• Difficulty focusing your eyes
• Difficulty swallowing

How does MSA affect the legs?

Multiple system atrophy is a rare neurodegenerative disorder that primarily affects the autonomic nervous system and movement control. While MSA primarily affects the autonomic nervous system, it can also lead to significant motor symptoms that affect the legs, which includes:

• Parkinsonian symptoms: Multiple system atrophy often starts with symptoms, such as slowness of movement, rigidity and tremors. These symptoms often affect the legs, causing stiffness and difficulty with movement.
• Balance and coordination: Problems with balance and coordination are common with multiple system atrophy, making it difficult to maintain an upright posture. This instability can affect the legs, leading to difficulties with walking and increasing the risk of falls.
• Gait abnormalities: Changes in gait, including a shuffling or hesitant walk, reduced stride length and difficulty initiating movement are common with multiple system atrophy.
• Orthostatic hypotension: Multiple system atrophy often results in orthostatic hypotension, a drop in blood pressure upon standing up. Symptoms include dizziness, lightheadedness and fainting, which can affect the legs' ability to support the body when standing.
• Muscle weakness: As the disease progresses, muscle weakness may develop in the legs. This weakness can contribute to difficulties with walking, climbing stairs and performing other activities that require leg strength.

Causes and risk factors

Why multiple system atrophy occurs involves various factors that contribute to its development. Scientists believe it results from a combination of genetic predispositions, environmental influences and other risk factors.

While the exact cause remains unclear, researchers have made significant strides in unraveling its origins.

Genetics plays a role in multiple system atrophy development. Certain genetic variations may increase a person's susceptibility to developing this disease, but they typically interact with other factors to trigger the disease. These genetic factors affect the functioning of proteins within cells, leading to abnormal protein buildup in the brain – a hallmark of multiple system atrophy.

Several factors may increase an individual's risk of developing multiple system atrophy. Age is a significant risk factor with the condition typically appearing in adulthood, usually around the ages of 50-60.

Additionally, men seem to be more susceptible to multiple system atrophy than women. Other risk factors include a history of certain neurological conditions, such as Parkinson's disease.

Diagnosing multiple system atrophy

Multiple system atrophy is a difficult disease to diagnose. Many symptoms also occur in other conditions, such as Parkinson’s disease or corticobasal degeneration.

A physical exam and digital imaging tests, such as an MRI and CT scan, may help your doctor determine if multiple system atrophy is a possible diagnosis.

Your doctor may perform a tilt table test to determine if you have a problem with blood pressure. They may also test automatic functions, such as:

• Bowel and bladder function
• Electrical signals of your heart with an electrocardiogram (ECG or EKG)
• Sweat test to check areas your body sweats
• A sleep study

Due to the challenges of diagnosing multiple system atrophy, many people aren’t diagnosed.

Multiple system atrophy treatment options

There’s no cure for multiple system atrophy or treatment available to slow its progression. The focus of treatment is managing the symptoms and making you as comfortable as possible.

Medications for controlling blood pressure and Parkinson’s-like symptoms, such as muscle stiffness, tremors and poor coordination are a key component of treatment.

If you are having bladder problems, your doctor may prescribe bladder medications or place a catheter in the later stages as multiple system atrophy advances.

Physical therapy, occupational therapy and speech therapy are often utilized to help improve daily life and help maintain independence. These therapies help with mobility, muscle weakness, communication and swallowing.