Primary Progressive Multiple Sclerosis (PPMS)

What is PPMS?

Primary progressive multiple sclerosis (PPMS) is a type of multiple sclerosis (MS) where your symptoms get worse over time.

It’s common for people diagnosed with MS to have relapsing symptoms (relapsing-remitting multiple sclerosis or RRMS), which are periods when their symptoms flare up. With PPMS, relapses can happen but they’re less common. This means that people diagnosed with PPMS will usually experience gradually worsening symptoms. This occurs slowly, with less frequent relapses or flare-ups.

Many people who have an RRMS (relapsing-remitting multiple sclerosis) diagnosis later develop another type of multiple sclerosis called secondary progressive multiple sclerosis (SPMS). PPMS and SPMS are sometimes grouped together as progressive MS, as they share similar symptoms. PPMS causes some inflammation and neurodegeneration. This is when nerve cells in your brain and nervous system lose function.

How common is PPMS?

Approximately 1 million people are living with MS in the United States. About 10% of people diagnosed with MS have PPMS.

What are the symptoms of PPMS?

The main symptom of PPMS is the gradual worsening of MS symptoms that could include:

• Vision changes.
• Tingling or numbness in your body.
• A squeezing feeling around your chest or abdomen.
• Feeling tired.
• Difficulty trying to pee or a feeling of urgency when you need to pee.
• An electrical shock down your back, arms or legs when you bend your neck forward.
• Trouble walking.
• Muscle stiffness or weakness.
• Brain fog or difficulty with memory and concentration.

Symptoms of PPMS aren’t entirely obvious at first but increase in severity over time. You may or may not have any symptom relapses or attacks with PPMS.

What causes PPMS?

The exact cause of MS is unknown. Research suggests it could be related to changes in your DNA (genetic predisposition) that make you more likely to have an autoimmune condition. Genetics only play a small part in MS, so your risk of passing the condition onto your future children is low.

Environmental factors can also play a role in developing MS. An example is exposure to bacteria or viruses.

If you have MS, your immune system mistakenly attacks healthy parts of your central nervous system, which causes symptoms.

What are the complications of PPMS?

Symptoms of PPMS can lead to the following complications:

• Muscle tightness (spasticity).
• Vision loss.
• Difficulty controlling your bladder.
• Sexual dysfunction.
• Difficulty with memory.
• Changes to your mood.

How is PPMS diagnosed?

There isn’t one test to confirm or rule out a diagnosis of PPMS. Your healthcare provider will make a diagnosis after collecting information from a variety of sources. After a physical exam and taking your medical history, your healthcare provider may offer tests, which can include:

• An MRI to look for damage on your brain and spinal cord.
• A lumbar puncture (spinal tap) to find characteristics of MS, like oligoclonal bands commonly found in MS, but which can also be seen in other conditions. They can represent evidence of inflammation involving the brain and/or spinal cord.
• Blood tests.
• An optical coherence tomography (OCT), which is a painless test that scans the nerves in the back of your eye (retina) to detect damage, possibly caused by MS.

What age do people get diagnosed with PPMS?

Most people receive a PPMS diagnosis in their 40s to 50s, but the condition can be present at any age.

How is PPMS treated?

Treatment for PPMS focuses on disease-modifying therapies (DMT) that change the course of the condition or symptom management.

An example of a DMT is ocrelizumab (Ocrevus®). This medication slows the speed of worsening symptoms. It’s effective in treating both RRMS and PPMS.

Symptom management decreases the severity of your symptoms to help you feel better. An example could include taking medications to regulate your bladder or physical therapy to strengthen your muscles. Treatment varies based on your symptoms.

Your healthcare provider will explain the possible side effects of each medication or treatment before you start it.

Note: There are many treatments available for MS but only a few medications are effective in PPMS. Healthcare providers think that’s because there’s less inflammation to target. That being said, there’s been tremendous research around MS and more treatments will likely be available.

Can PPMS be prevented?

There’s no known way to prevent MS. To reduce relapses of symptoms, continue treatment as directed and avoid triggers in your environment.

What can I expect if I have PPMS?

PPMS can have an impact on your daily life. As your symptoms progress, you may need to make adjustments to keep yourself safe and prevent injury. For example, if you’re having trouble getting around on your own, talk to your healthcare provider about mobility devices like a wheelchair to help you gain more independence.

While PPMS can impact you physically, a chronic condition can affect your mental health as well. Some people find comfort in talking with a mental health professional, as the condition can affect your mood.

While there isn’t a cure for PPMS, research is ongoing to learn more and find new treatment options to help you feel better.

How fast do symptoms of PPMS progress?

Each person diagnosed with PPMS has a different outlook. Some symptoms can take years to progress, while others may happen more quickly. If you notice changes to your symptoms, make sure to discuss those changes with your healthcare provider.

What is the life expectancy for someone with PPMS?

PPMS doesn’t directly affect your life expectancy.

When should I see a healthcare provider?

Contact your healthcare provider if you have worsening symptoms of PPMS that affect your daily life or if you have complications like paralysis, ongoing pain or numbness in your arms and legs. As PPMS can affect your balance and coordination, contact your provider and/or emergency services if you fall and injure yourself.

What questions should I ask my doctor?

• Do I have PPMS or another form of MS?
• How do I manage my symptoms?
• Do you recommend physical or occupational therapy?
• Are there side effects of the medication you recommend?
• Do I need to use a wheelchair or other mobility device?

A note from Cleveland Clinic

Primary progressive multiple sclerosis (PPMS), like all types of multiple sclerosis, can have a significant impact on your life. Treatment is available to slow the progression of your symptoms. You may not notice your symptoms at first, but they can get worse over time. You may need to use mobility devices like a wheelchair to help you gain more independence. Your healthcare provider can help you manage your symptoms so you can lead a full life.