What is primary progressive multiple sclerosis (PPMS)?
In multiple sclerosis, the immune system attacks the brain, spinal cord and optic nerves. These make up the central nervous system, which controls everything we do. Damage from the attack disrupts signals to and from the brain and causes the symptoms of MS.
Primary progressive multiple sclerosis (PPMS) is a type of MS. Ten to 15 percent of people with MS have this type. If you have PPMS, you will experience gradually worsening neurologic symptoms and an accumulation of disability. You will not have relapses, or attacks, early in the disease course. You also will not have remissions.
PPMS can be further characterized as either active (with an occasional relapse and/or evidence of new magnetic resonance imaging (MRI) activity over a specified period of time) or not active, as well as with progression (evidence of disability accrual over time, with or without relapse or new MRI activity) or without progression.
This graphic shows the kinds of disease activity that can occur in PPMS. However, each person's experience with PPMS will be unique. As you can see in the graph, you might have brief periods when the disease is stable, with or without a relapse or new MRI activity. You may also experience periods of increasing disability with or without new relapses or lesions on MRI.
How do I know if my disease course is stable?
Disease activity and progression can be evaluated by neurologic examination and MRI. Monitoring your disease course at different points in time helps you and your MS care provider have important conversations about your treatment options and prognosis. For example:
- If you have PPMS that is active, with new MRI activity or relapses, your conversation with your MS care provider could be about starting treatment with a disease-modifying therapy to reduce the risk of a relapse, as well as rehabilitation to help improve function and mobility.
- If you have PPMS that is stable without activity (no new MRI activity or relapses) or progression, the conversation with your MS care provider could include the role of rehabilitation to help you maintain function, as well as other symptom management strategies that you may need.
- If you have PPMS that is not active (no new MRI activity or relapses) but is progressing with increasing accumulation of disability, the conversation with your MS provider could focus on the rehabilitation strategies that can help you maintain function and keep you safe and independently mobile.
How does PPMS differ from the other disease courses?
Although there is a lot of variability among people with PPMS, as a group they differ in several ways from people with relapsing forms of MS.
- PPMS can take longer to diagnose. It requires a minimum of 12 months of symptom progression. RRMS can be diagnosed much more quickly.
- Only one DMT is FDA-approved to treat PPMS. More than 20 DMTs are approved for relapsing forms of MS. This means that people with PPMS have fewer treatment options and that there are no treatments to completely prevent disease progression/disability accumulation.
- The average age of onset is approximately 10 years older in PPMS than in relapsing MS.
- People with PPMS tend to experience more problems with walking and more difficulty remaining in the workforce.
- In general, people with PPMS may require more assistance with everyday activities.
Common signs and symptoms of primary progressive MS
PPMS is characterized by a fairly steady, gradual change in functional ability over time without any remissions. But one person’s PPMS symptoms may progress differently or more rapidly than someone else’s. Because spinal cord lesions are more common than brain lesions in PPMS, problems with walking are very common. Other symptoms of PPMS include:
- Balance problems
- Bladder and bowel issues
- Electric-shock sensations down back
- Mood changes
- Muscle weakness
- Problems with cognition (learning and memory or information processing)
- Sexual problems
- Vision problems
Read more about these and other MS symptoms.
Diagnostics and tests for PPMS
At this time, there are no symptoms, physical findings or laboratory tests that can, by themselves, determine if you have MS. Several strategies are used to determine if you meet the criteria for a diagnosis of MS and to rule out other possible causes of your symptoms. These strategies include:
- A careful medical history
- A neurologic exam
- Various tests including MRI, spinal fluid analysis and blood tests to rule out other conditions
The only definitive way to differentiate PPMS from other forms of MS is by reviewing your history of symptoms. The following criteria must be met in order to confirm the diagnosis of PPMS:
- One year of disease progression (worsening of neurological function without remission) AND
- Two of the following:
- A type of lesion in the brain that is recognized by experts as being typical of MS
- Two or more lesions of a similar type in the spinal cord
- Evidence in the spinal fluid of oligoclonal bands or an elevated IgG index, both of which are indicative of immune system activity in the CNS
Meeting these criteria can take a fairly long time, particularly if the person has only recently begun to experience neurologic symptoms.
PPMS experiences and stories
Read firsthand accounts in the Society’s blog of what it’s like to be diagnosed with and to live with PPMS.
We are pursuing all promising research paths and collaborating worldwide to drive progress in research in progressive MS, for which few therapies exist. Learn more about progressive MS research.