Patient Resources

Primary Progressive Multiple Sclerosis

What is primary progressive multiple sclerosis?

Multiple sclerosis (MS) is a neurodegenerative disease. It interferes with your brain's ability to control your body. It can be disabling.

There are 4 main types of MS:

Relapsing-remitting MS
Primary progressive MS
Secondary progressive MS
Progressive-relapsing MS

Each type may be mild, moderate, or severe. Each type affects people differently.

With primary progressive multiple sclerosis (PPMS), nervous system problems get worse from the beginning. There are no symptom flare-ups (relapses or attacks). And there is no recovery (remission). How fast the disease gets worse may vary. There can be times when things are stable. And there can be periods of short-term (temporary) minor improvements. But nervous system problems steadily get worse.

Men and women are evenly affected by this type of MS.

About 1 in 10 people diagnosed with MS have PPMS. On average, people with PPMS start having symptoms between ages 35 and 39.

What causes primary progressive MS?

Healthcare providers don’t know the exact cause of MS. It may develop when your immune system attacks a substance called myelin.

Myelin acts as a type of insulation for your nerve cells. This process can lead to damage in and around the nerves in your brain and spinal cord. It can also damage nerves used for vision.

What are the symptoms of primary progressive MS?

Symptoms can be a bit different for each person. Symptoms can include:

Pain, such as pain in the legs and feet, back pain, and muscle spasms
Electric-shock sensations that run down the back and limbs when the neck is bent (Lhermitte sign)
Trouble walking
Vision problems
Muscle weakness
Trouble staying balanced
Paralysis
Numbness
Prickling feelings
Dizziness
Shakiness
Trouble thinking clearly
Mood changes
Depression
Sexual problems
Trouble with bowel and bladder control

How is primary progressive MS diagnosed?

Methods that your healthcare provider may use to diagnose MS include:

Discussion. Your healthcare provider will talk with you about your symptoms.
Physical exam. This is done to see how your nerves and muscles are working.
MRI scans of your brain and spinal cord. These images will help your healthcare provider look for signs of damage that suggest MS.
Optical coherence tomography. This test measures nerve fibers in the retina.
Blood work. This is to look for infections or health conditions that can look like MS.
Spinal tap (lumbar puncture). Your healthcare provider removes a sample of spinal fluid to check for signs of MS.
Visual evoked potentials. This test is done to see how well your optic nerves are working.

After the general diagnosis of MS, the diagnosis of PPMS is based almost entirely on your symptom history. Because of this, it may take time for the PPMS diagnosis to be made.

How is primary progressive MS treated?

Ocrelizumab (ocrevus) is currently the only medicine approved to treat primary progressive MS.

Your healthcare provider will also try to provide treatments that ease symptoms and improve your quality of life. These may address problems like spasticity, depression, sexual problems, and extreme tiredness (fatigue). These treatments can include medicines, physical therapy, counseling, and surgery.

How can I prevent primary progressive MS?

Experts don't know how to prevent MS or the PPMS type of MS. Some people limit MS relapses by staying away from certain triggers, such as stress and overheating.

How do I manage primary progressive MS?

Physical and occupational therapy may be helpful. For example, therapists may teach you exercise strategies and how to manage new symptoms that develop. Your healthcare provider will also likely want to meet with you on a regular basis to check your disease.

Regular exercise and plenty of sleep may also help. If your body temperature goes up, it may make your symptoms worse for a short time. So it’s best to not do things that could lead to overheating. This includes spending long periods of time in the sun or overexerting yourself.

Managing MS can be difficult, frustrating, and frightening. If you have feelings of anxiety or depression, ask your healthcare provider for help. Support groups for patients and families, and individual therapy, can help people deal with the challenges and changes that people with MS and their loved ones face.

Key points about primary progressive multiple sclerosis

Multiple sclerosis (MS) is a disease that interferes with your brain's ability to control your body.
Primary progressive MS (PPMS) is a type of MS. It has no symptom flare-ups. And there is no recovery. Nervous system problems get worse.
Symptoms may include pain, trouble walking, vision problems, and muscle weakness.
Your healthcare provider may give you a medicine to slow the disease as well as to help manage your symptoms and improve your quality of life.
Physical and occupational therapy may be help you manage PPMS. Regular exercise and plenty of sleep may also help.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

Know the reason for your visit and what you want to happen.
Before your visit, write down questions you want answered.
Bring someone with you to help you ask questions and remember what your provider tells you.
At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new directions your provider gives you.
Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are and when they should be reported.
Ask if your condition can be treated in other ways.
Know why a test or procedure is recommended and what the results could mean.
Know what to expect if you do not take the medicine or have the test or procedure.
If you have a follow-up appointment, write down the date, time, and purpose for that visit.
Know how you can contact your healthcare provider if you have questions after hours and during weekends.