Empowering people affected by MS to live their best lives

Understanding Primary Progressive Multiple Sclerosis (PPMS)

Multiple sclerosis is an unpredictable disease of the central nervous system that disrupts the flow of information within the brain and between the brain and body. Primary progressive multiple sclerosis (PPMS) is a type of MS. About 10% to 15% 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 early in the disease course. You also will not have remissions.PPMS can be 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 graph shows the kinds of disease activity that can occur in PPMS. However, each person's experience with PPMS will be unique. As the graph shows, 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.

Monitoring the Disease Course

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 (DMT) 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.

Ask an MS Expert: Progressive MS

How PPMS Differs From Other Disease Courses

Although every person’s experience with PPMS is different, PPMS is unlike relapsing forms of MS in a number of ways:

PPMS can take longer to diagnose. It requires a minimum of 12 months of symptom progression. RRMS can be diagnosed much more quickly.
Currently, 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.
The average age of onset is about 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 ability over time without any remissions. But one person’s PPMS symptoms may progress differently or more rapidly than someone else’s. Spinal cord lesions are more common than brain lesions in PPMS, so 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
Paralysis
Problems with cognition (learning and memory or information processing)
Sexual problems
Tremors
Vision problems

Read more about these and other MS symptoms.

Diagnosing 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 together 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

Unlike relapsing forms of MS, primary progressive MS is characterized by a fairly steady, gradual change in functional ability over time — most often related to walking — without any relapses.Due to this basic difference in the disease course, different criteria are used to make an accurate diagnosis of PPMS than for other types of MS. The criteria for a diagnosis of PPMS are:

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 central nervous system

Meeting these criteria can sometimes take a fairly long time, particularly if you have only recently begun to experience neurologic symptoms. Several studies have suggested that PPMS may take 2 to 3 years longer to diagnose than relapsing-remitting MS.

Treating PPMS — Modifying the Disease Course

Disease-modifying therapies are medications that work primarily by reducing inflammation in the central nervous system. They do not work as well in a disease course that is characterized by nerve degeneration rather than inflammation. For this reason, they have not been shown to be effective in progressive forms of the disease unless a person demonstrates relapses or MRI activity caused by inflammation.Several clinical trials are ongoing for progressive forms of MS, including PPMS. Read more about clinical trials for MS.In addition to treatment with a disease-modifying therapy, other symptom management and rehabilitation strategies may help people with PPMS manage the disease.

Managing symptoms

Regardless of the course of MS you are experiencing, proactive symptom management is essential to maintaining comfort and productivity, and enhancing quality of life.

Rehabilitation

Rehabilitation specialists have an important role from the time of diagnosis helping you function optimally in spite of your MS, including enhancing mobility and promoting safety and independence.

Healthy living

You can achieve better health and wellness, including emotional well-being, even with a chronic illness or disability.

Research

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 MS research.

Although every person’s experience with PPMS is different, PPMS is unlike relapsing forms of MS in a number of ways:

PPMS can take longer to diagnose. It requires a minimum of 12 months of symptom progression. RRMS can be diagnosed much more quickly.
Currently, 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.
The average age of onset is about 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

Balance problems
Bladder and bowel issues
Electric-shock sensations down back
Mood changes
Muscle weakness
Paralysis
Problems with cognition (learning and memory or information processing)
Sexual problems
Tremors
Vision problems

Read more about these and other MS symptoms.

Diagnosing PPMS

A careful medical history
A neurologic exam
Various tests including MRI, spinal fluid analysis and blood tests to rule out other conditions

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 central nervous system

Treating PPMS — Modifying the Disease Course

Managing symptoms

Regardless of the course of MS you are experiencing, proactive symptom management is essential to maintaining comfort and productivity, and enhancing quality of life.

Rehabilitation

Healthy living

You can achieve better health and wellness, including emotional well-being, even with a chronic illness or disability.

Research

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 MS research.