Key takeaways
Primary progressive multiple sclerosis (PPMS) worsens steadily from the first symptoms, without remissions. Symptoms may stall, but lost function typically does not return.
Walking problems and leg weakness are common early signs, often getting more noticeable over about 2 years. PPMS affects about 10 to 15% of people with multiple sclerosis and often increases daily assistance needs.
Ocrelizumab (Ocrevus) is the only Food and Drug Administration (FDA)-approved drug for PPMS. Exercise, stretching, physical therapy, and mental health support may help manage symptoms and improve quality of life.
Primary progressive multiple sclerosis (PPMS) is a unique type of MS. PPMS progresses from the time you notice your first (or primary) symptoms. Unlike with other types of MS, you will not have remissions, or periods of reduced disease activity. You likely won’t have relapses, or periods of increased disease activity, early in the disease course.
PPMS isn’t very common. About
of people with MS have it.
If you have PPMS, there are more instances of walking disability than other symptoms when compared with people who have other types of MS.
The diagnosis of progressive-relapsing MS (PRMS) is now considered primary progressive MS.
Other types of MS
The types of MS, also called courses, are defined by how they affect your body.
Other types of MS include:
relapsing-remitting MS (RRMS)
secondary progressive MS (SPMS)
clinically isolated syndrome (CIS)
Each type has different treatments, with many therapies overlapping. The severity of their symptoms and long-term outlooks also vary.
CIS is a newly defined type of MS. CIS happens when you have a single period of neurologic symptoms that lasts for at least 24 hours.
The outlook for people with PPMS is different for everyone and unpredictable.
Compared with people who have other forms of MS, people with PPMS
more problems with walking and are more likely to leave the workforce early. They’re more likely to require additional assistance with daily life and activities.
Symptoms may become more noticeable over time, especially as you get older and begin to lose certain functions in organs like your bladder, bowels, and genitals due to age and PPMS.
Four
characterize PPMS over time:
Active with progression describes PPMS with worsening symptoms and relapses or with new MRI activity. Increasing disability also typically occurs.
Active without progression describes PPMS with relapses or MRI activity but no increasing disability.
Not active with progression describes PPMS with no relapses or MRI activity but with increasing disability.
Not active without progression describes PPMS with no relapses, MRI activity, or increasing disability.
A key characteristic of PPMS is the lack of remissions.
Even if a person with PPMS sees their symptoms stall — meaning they don’t experience worsening disease activity or an increase in disability — their symptoms do not improve.
With PPMS, people don’t regain the function they may have lost.
Here are some main differences between PPMS and RRMS:
RRMS is the most common type of MS, accounting for about
of diagnoses, while PPMS is one of the rarest.
RRMS is
as common in females than males while PPMS is equally common in females and males.
Episodes of new symptoms are
in RRMS than in PPMS.
During a remission in RRMS, you may not notice any symptoms at all, or you may have just a few symptoms that aren’t as severe.
Typically, more brain lesions appear on brain MRIs with RRMS than with PPMS, if untreated.
RRMS tends to be diagnosed much earlier in life than PPMS, in around the 20s and 30s, as opposed to the 40s and 50s with PPMS.
Some main differences between PPMS and SPMS include:
Many people with MS first receive a diagnosis of RRMS.
SPMS
as a diagnosis of RRMS that eventually becomes more severe over time without any remissions or improvements in symptoms.
SPMS is often the second stage of an MS diagnosis.
PPMS is an initial diagnosis on its own.
PPMS affects everyone differently.
Common early symptoms of PPMS include weakness in your legs and trouble walking. These symptoms typically become more noticeable over a period of 2 years.
Other common symptoms of PPMS include:
stiffness in the legs
problems with balance
pain
weakness and tiredness
trouble with vision
bladder or bowel dysfunction
depression
fatigue
numbness, tingling, or both in different parts of the body
It can be difficult to differentiate between RRMS and PPMS because they have similar symptoms. Symptoms that become worse consistently over 1 year may be a sign that a person has PPMS.
A person with PPMS may have:
brain lesions visible on an MRI scan
two or more spinal cord lesions
elevated levels of immune proteins or antibodies in their spinal fluid
The exact cause of PPMS, and MS in general, isn’t yet known.
The most common theory is that MS begins when the immune system starts attacking the central nervous system. This results in a loss of myelin, the protective covering around nerves in the central nervous system.
While doctors don’t believe that PPMS can be inherited, it
a genetic component. Some experts believe a virus or a toxin in the environment when combined with a genetic predisposition for MS may trigger the condition.
Each type of MS has a different outlook and treatment needs.
There’s no specific test that provides a PPMS diagnosis. Doctors often have difficulty diagnosing PPMS compared with other types of MS and other progressive conditions.
This is because a neurological issue needs to have progressed for 1 or 2 years for doctors to confirm a PPMS diagnosis.
Other conditions with symptoms similar to PPMS include:
an inherited condition that causes stiff, weak legs
a vitamin B12 deficiency that causes similar symptoms
Lyme disease
viral infections, such as human T-cell leukemia virus type 1 (HTLV-1)
forms of arthritis, such as spinal arthritis
a tumor near the spinal cord
To diagnose PPMS, a doctor may:
evaluate your symptoms
review your neurological history
conduct a physical examination focusing on your muscles and nerves
conduct an MRI scan of your brain and spinal cord
perform a lumbar puncture to check for signs of MS in spinal fluid
conduct evoked potential (EP) tests to identify the specific type of MS (EP tests stimulate sensory nerve pathways to determine electrical activity of the brain)
Ocrelizumab (Ocrevus) is the only drug approved by the Food and Drug Administration (FDA) to treat PPMS. It helps limit nerve degeneration.
Some medications treat specific symptoms of PPMS, such as:
muscle tightness
pain
fatigue
bladder and bowel problems
While many
disease-modifying therapies (DMTs)
and steroids are approved by the FDA for relapsing forms of MS, they don’t specifically treat PPMS.
Several new
are being developed for PPMS to help reduce inflammation that specifically attacks your nerves.
Some of these also help address damage and repair processes that affect your nerves. These treatments may be able to help restore myelin around your nerves damaged by PPMS.
A
found that ibudilast, one possible treatment, decreased brain atrophy in participants with progressive MS, but it didn’t reduce the number of new lesions. It may have some ability to treat inflammation in PPMS. Trials are still ongoing.
Another treatment in clinical trials called
has been studied for allergies and may target mast cells involved in allergic reactions. It shows promise as a treatment for PPMS, too.
A new treatment called
may help disrupt the immune response that damages the myelin coating of nerves that occurs in MS. Trials for its use in PPMS and RRMS are still ongoing.
These three treatments are still in the early stages of development and research.
People with PPMS can relieve symptoms with exercise and stretching to:
stay as mobile as possible
manage weight
increase energy levels
Some other strategies that can help manage your PPMS symptoms and maintain your quality of life include:
eating a balanced and nutritious diet
staying on a regular sleep schedule as much as possible
going to physical or occupational therapy, which can teach you strategies for increasing mobility and managing symptoms
If you’re living with PPMS, it’s important to find sources of support.
Living with a chronic illness can take an emotional toll. If you’re experiencing ongoing feelings of sadness, anger, grief, or other difficult emotions, let your healthcare team know. They may refer you to a mental health professional who can help.
You can also look for a mental health professional on your own. For example, the American Psychological Association offers a
to find psychologists throughout the United States.
also offers a treatment referral helpline.
You may find it helpful talking with other people who are living with MS. Consider looking into support groups and other communities, either in person or online, like
.
The
offers a service to help you find local support groups in your area. The organization also has a peer-to-peer connection program run by trained volunteers who live with MS.
What is the life expectancy of someone with primary progressive MS?
The average life expectancy for someone with PPMS is 71.4 years, according to a
in Norway that followed participants for 60 years. Most people with MS have a
life expectancy. However, people with PPMS may have a greater risk of life threatening complications.
At what age do most people receive a diagnosis of primary progressive MS?
People with PPMS typically receive a diagnosis in their
. It can take longer to receive a diagnosis of PPMS than RRMS.
What does primary progressive MS mean?
PPMS is a type of MS that progresses from the time you notice your first (or primary) symptoms. People with PPMS do not experience remissions, or periods of reduced disease activity.
It’s possible to have a high quality of life with PPMS when you work with a doctor to figure out the best treatments as well as lifestyle and dietary strategies for you.
There’s no current cure for PPMS, but treatment makes a difference. Although the condition is progressive, people can experience periods of time when symptoms don’t actively worsen.
If you’re living with PPMS, a doctor can recommend a treatment plan based on your symptoms and general health.
Developing positive lifestyle habits and staying connected to sources of support can also help you maintain your quality of life and overall well-being.