Multiple system atrophy (MSA) is a rare, progressive, and fatal neurological disorder that causes nerve cells in the brain and spinal cord to deteriorate over time. As nerve cells die, it leads to problems with movement, coordination, balance, and autonomic functions such as breathing, digestion, and blood pressure control.
Although there is no cure for MSA, UPMC neurology experts can help you manage symptoms with medication, rehabilitation, supportive care, and mental health counseling.
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What Is Multiple System Atrophy (MSA)?
Multiple system atrophy (MSA) is a rare, progressive, and fatal neurological disorder that causes nerve cells in the brain and spinal cord to deteriorate and die over time. As nerve cells die, this leads to problems with movement, balance, coordination, and autonomic functions such as breathing, digestion, and blood pressure control.
MSA was previously known as Shy-Drager syndrome, olivopontocerebellar atrophy (OPCA), and striatonigral degeneration.
What are the types of MSA?
- Cerebellar type MSA (MSA-C) — Causes problems with balance, coordination, swallowing, speech, and eye movement.
- Parkinsonian type MSA (MSA-P) — Causes similar symptoms to Parkinson’s disease, such as slow movements, stiffness, and tremors, along with problems with balance, coordination, and the body’s autonomic functions, such as digestion, breathing, sweating, and blood pressure control.
Patients may have features of both types.
How does MSA differ from Parkinson’s disease?
MSA and Parkinson’s disease have similar symptoms, especially at first.
However, they are distinct conditions with several differences, including:
- MSA gets worse more quickly than Parkinson’s disease.
- Autonomic symptoms develop faster and are more severe with MSA.
- Tremors are more common and severe with Parkinson’s disease.
- Some common Parkinson’s disease treatments won’t work for MSA.
How common is MSA?
MSA is rare, affecting approximately three to five people per 100,000 in the U.S.
What causes MSA?
Researchers aren’t sure what causes MSA. However, they suspect it may be linked to a buildup of alpha-synuclein protein, which causes nerve cell damage in the brain.
Complications of MSA
MSA may cause life-threatening complications, including:
- Breathing problems.
- Incontinence.
- Pneumonia.
- Urinary tract infections.
- Sepsis.
- Sudden death.
- Swallowing difficulties.
What Are the Signs and Symptoms of MSA?
MSA affects movement, autonomic functions, emotions, and thinking. Most people develop movement-related symptoms before other symptoms.
Symptoms may vary depending on which area of the brain is affected.
Movement-related symptoms
Movement-related symptoms may be different depending on the type of MSA you have.
MSA-C movement symptoms
- Abnormal eye movements.
- Loss of coordination.
- Tremors that worsen when you attempt to use the affected limb.
- Wide steps when walking.
MSA-P movement symptoms
- Frequent falls.
- Hunched-over posture.
- Slow movements.
- Speech changes, such as mumbling or slurring.
- Stiffness.
Autonomic symptoms
Autonomic symptoms occur when damage to your nerve cells disrupts your body’s ability to perform involuntary functions, including:
- Constipation.
- Decreased sweating.
- Dry mouth.
- Loss of bladder or bowel control.
- Sexual dysfunction.
- Slow digestion.
- Sudden drops in blood pressure when standing up from a seated or lying-down position.
Cognitive and emotional symptoms
Cognitive and emotional symptoms may include:
- Anxiety.
- Depression.
- Difficulty controlling emotions.
- Difficulty with thinking or concentration.
- Unstable emotions, including laughing or crying at inappropriate times.
When should I see a doctor about my MSA symptoms?
If you have symptoms of MSA, you should schedule an appointment with your doctor as soon as possible. Your doctor may refer you to a neurologist who specializes in treating neurodegenerative disorders.
How Do You Diagnose MSA?
The only way to confirm an MSA diagnosis is to check for a buildup of alpha-synuclein protein in the brain after death. People with MSA are often diagnosed with Parkinson’s disease first and receive a revised diagnosis as the condition progresses.
To diagnose MSA, your doctor will perform physical and neurological examinations and review your symptoms and medical history. Your doctor may also order tests to help make a diagnosis or rule out other problems.
What to expect during your visit
During your visit, your doctor will:
- Ask you about your symptoms.
- Order tests.
- Perform a physical and neurological exam.
- Review your medical history.
Tests to diagnose MSA
Your doctor may order tests to provide information or rule out other problems, including:
- Blood and urine tests — Checks for abnormalities and rules out other conditions.
- Cerebral spinal fluid analysis (spinal tap or lumbar puncture) — Involves inserting a needle into your lower back to collect a sample of cerebrospinal fluid (CSF) for analysis in a lab.
- DaT scan - A special brain scan that is sometimes used to diagnose Parkinson's disease.
- Electromyography (EMG) — Sometimes called an electromyogram, an EMG allows a doctor to record and study the electrical activity of your muscles.
- Genetic tests — Blood tests to look for signs of genetic changes.
- Magnetic resonance imaging (MRI) — Creates images of your brain and spine using contrast dye to look for damaged or abnormal areas.
- Nerve conduction study (NCS) — Measures the speed of electrical activity passing through your nerves.
- Neurological examination — A noninvasive test to check your reflexes, balance, movement, memory, and ability to think.
- Neuropsychological examination — Checks your ability to think, reason, and remember.
- Skin biopsy — Involves taking a small sample of your skin to look for proteins associated with MSA.
MSA prognosis
As MSA symptoms worsen, most people need to use a wheelchair and eventually are confined to bed. Some people need breathing support, a feeding tube, or surgical procedures to assist with bowel and bladder function.
What is the life expectancy of someone with MSA?
Most people with MSA live six to 10 years after diagnosis. Some people may live longer.
Can you recover from MSA?
MSA is a lifelong condition. There is no cure, and symptoms get progressively worse over time.
How Do You Treat MSA?
There is no cure for MSA. The goals of MSA treatment are to manage symptoms, reduce your risk of complications, and improve quality of life. Treatment options may include:
Medication
Your doctor may recommend medications to manage symptoms, including:
- Antidepressants — Treat depression and anxiety.
- Baclofen — Treats muscle stiffness and spasms.
- Benzodiazepines — Treat muscle spasms or abnormal movements.
- Carbidopa-levodopa — Treats Parkinsonism symptoms.
- Glycopyrrolate or scopolamine — Treats drooling.
- Mirabegron or oxybutynin — Treats urinary symptoms.
Supportive care
Your doctor may prescribe treatments to manage symptoms and complications, including:
- Feeding and hydration support — If the muscles that help you chew and swallow are affected, IV fluids will prevent dehydration. Eventually, you may need to receive nutrition through a gastrostomy tube that is surgically placed in your abdomen.
- Mental health counseling — Counseling can help you and your loved ones cope with the challenges of an MSA diagnosis and manage mental health conditions, such as anxiety and depression.
- Respiratory therapy — If the muscles that control your breathing are affected, your doctor may recommend medications or mechanical ventilation to help you breathe.
Rehabilitation
You may receive different types of rehabilitation therapy to help you maintain strength, mobility, and independence. Rehabilitation treatment may include:
- Physical therapy — Maintains strength, balance, and mobility using exercises, stretching, massage, electrical stimulation, and other techniques.
- Occupational therapy — Teaches techniques and strategies to help you perform everyday activities, such as bathing, dressing, cooking, and grooming, sometimes with the assistance of a cane, brace, walker, or wheelchair.
- Speech and swallowing therapy — Helps you maintain the ability to swallow, chew, and speak.
Clinical trials
You may be eligible to participate in a clinical trial studying new treatments for MSA. Your doctor will let you know if you qualify for a clinical trial.
Why Choose UPMC for MSA Care?
When you choose UPMC for MSA care, you will receive:
- Multidisciplinary care — Our team of neurologists, physical therapists, and speech therapists will work with you and your loved ones to develop a customized treatment plan that optimizes your quality of life and reduces your risk of complications.
- Access to advanced clinical trials — Our team participates in national, multicenter clinical trials evaluating new treatment options, allowing you to contribute to research advancing care for MSA.
- Seamless, ongoing treatment — If you are diagnosed with MSA, our team will develop a plan for your ongoing care that reflects your goals, preferences, and wishes.