Multiple System Atrophy

Multiple system atrophy (MSA) is a rare neurological disease that causes certain brain areas to deteriorate. Over time, this disrupts abilities and functions handled by those brain areas. This disease is ultimately fatal.

MSA is a new term that combines features of three older condition names. Those conditions are:

• Shy-Drager syndrome.
• Sporadic olivopontocerebellar atrophy.
• Striatonigral degeneration.

Experts introduced the name multiple system atrophy after noticing that the above conditions had several features in common. The symptoms depend on the brain areas affected, which is why the symptoms can have different forms and combinations.

After recognizing MSA as a specific disease, experts further defined it by classifying it into two types based on symptoms:

• MSA-C. The C in this name is for “cerebellar.” This type of MSA affects your ability to coordinate your movements, causing symptoms like ataxia. That symptom is the most prominent feature, but people with this type also experience autonomic dysfunction and falls.
• MSA-P. The P in this name is for “parkinsonism.” This type of MSA has symptoms that are similar to what happens with the parkinsonism family of movement disorders. Parkinsonism may be more prominent in the beginning, but autonomic features can also happen along with ataxia.

Who does multiple system atrophy affect?

MSA affects adults over age 30. The symptoms are most likely to start between ages 50 and 59. The condition doesn’t affect people differently depending on sex.

How common is multiple system atrophy?

Multiple system atrophy is a rare condition. Experts estimate an average of 0.6 to 0.7 new cases per 100,000 people yearly. The estimated number of total cases is between 3.4 and 4.9 per 100,000 people.

How does multiple system atrophy affect my body?

MSA causes deterioration in different brain areas. The symptoms depend on the areas affected. The parts of your brain most commonly affected include:

• Basal ganglia. These are structures near the center of your brain that link many different areas of your brain together. They create a critical network that allows different parts of your brain to work cooperatively.
• Brainstem. Your brainstem is responsible for managing many of your body’s key autonomic processes. You need these elements to stay alive, but your body handles them automatically without you thinking about them. They include breathing, heart rate, blood pressure, etc.
• Cerebellum. This structure at the back of your head helps coordinate movements. It also works cooperatively with other brain areas. Researchers are still learning what it does, but some evidence suggests it even plays a role in other things like emotions and decision-making.

MSA symptoms can vary depending on which areas of your brain this condition affects. As the affected areas deteriorate, you’ll have more and more difficulty with the abilities those areas control. For example, deterioration of your brainstem causes problems with autonomic processes like blood pressure.