What to Know About the Symptoms of Primary Progressive Multiple Sclerosis (PPMS)

The outlook for people with PPMS is different for everyone and unpredictable.

Compared with people who have other forms of MS, people with PPMS may experience 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 modifiers 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 85% of diagnoses, while PPMS is one of the rarest.
• RRMS is three times as common in females than males while PPMS is equally common in females and males.
• Episodes of new symptoms are more common 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 often begins 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 may have 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 treatments 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 2021 study 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 masitinib 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 fenebrutinib 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 search tool to find psychologists throughout the United States. MentalHealth.gov 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 Bezzy MS.

The National MS Society 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 2017 study in Norway that followed participants for 60 years. Most people with MS have a typical 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 40s or 50s. 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.