A look at multiple system atrophy
DEAR MAYO CLINIC: Seven years ago, I was diagnosed with multiple system atrophy — also known as Shy-Drager syndrome. I’m wondering if there are any new treatments or procedures for my ailment. I am 78 years old. Is there any benefit in being reevaluated?
The basic advice is to see your doctor if you have new symptoms or symptoms that have worsened. Your doctor may be able to suggest management strategies to help you cope with this condition.
Multiple system atrophy — that is the preferred name — is a degenerative neurological disorder. It’s complex and characterized by a constellation of neurologic symptoms related to your autonomic nervous system and certain motor systems. The autonomic nervous system controls the body functions you don’t have to think about, such as blood pressure, heart rate, bowel and bladder function, sweating and digestion.
The primary sign of multiple system atrophy is usually a drop in blood pressure when you stand. This causes dizziness, lightheadedness and may even result in passing out. Other common symptoms include:
• Inadequate sweating
• Incontinence and constipation
• Erectile dysfunction
• Motor disorders that appear similar to Parkinson’s symptoms
• Rigid muscles that make bending arms and legs difficult
• Restless sleep, including violent thrashing during dreams
• Difficulty breathing and swallowing
• Slurred speech
• Poor balance and incoordination
Most patients have a combination of these symptoms. As the disease progresses, they may have all of these symptoms. Dementia or a loss of cognitive ability are not usually present, which differentiates multiple system atrophy from some other neurological conditions.
By managing symptoms, we work with patients to keep them as active and comfortable as possible. Eventually, the condition is fatal. Most often, the cause of death is due to disordered breathing or pneumonia.
In some patients, the symptoms worsen quickly. Around 80 percent of people with multiple system atrophy are disabled within five years after the first motor symptoms occur. In other patients, the disease progresses slowly. They may live another 15 or even 20 years.
Unfortunately, we don’t know how to determine the course of the disease for an individual, or how to slow its progression.
Researchers have learned that some forms of multiple system atrophy run in families. What triggers the disorder or increases the risk is also unknown at this time, although we do know the disease usually occurs in people older than age 50.
There are no guidelines on when patients with multiple system atrophy should be reevaluated by their physician, because the course of the disease varies for each patient. Since your diagnosis, there have been improvements in how we manage common symptoms, especially low blood pressure when standing and incontinence. If you haven’t talked with your doctor about your symptoms for more than a year, it would be a good idea to have that discussion.
Treatment should be carefully tailored to each patient’s needs, and many options are available.
Occasionally, a pacemaker is implanted to help regulate the heart. Other treatments include placement of a permanent feeding tube into the stomach through a small skin incision, or a tracheotomy, which is a surgery to open the windpipe and assist with breathing. — Paola Sandroni, Ph.D., M.D., Neurology, Mayo Clinic, Rochester.
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