Multiple sclerosis (MS) is a chronic autoimmune disorder that affects the optic nerves, spinal cord, and brain.
People who are diagnosed with MS often have very different experiences. This is especially true for those diagnosed with primary progressive multiple sclerosis (PPMS), one of the rarer types of MS.
PPMS is a unique type of MS. It doesn’t involve as much inflammation as forms of MS that relapse.
The main symptoms of PPMS are caused by nerve damage. These symptoms occur because nerves aren’t able to send and receive messages to each other properly.
If you have PPMS, there are more instances of walking disability than other symptoms, when compared with people who have other types of MS.
PPMS isn’t very common. It affects about 10 to 15 percent of those diagnosed with MS. PPMS progresses from the time you notice your first (primary) symptoms.
Some types of MS have periods of acute relapses and remissions. But the symptoms of PPMS get more noticeable slowly but steadily over time. People with PPMS can also have relapses.
PPMS also causes neurological function to decline much faster than in other MS types. But the severity of PPMS and how fast it develops depends on each case.
Some people may have continued PPMS that gets more severe. Others may have steady periods without flare-ups of symptoms, or even periods of small improvements.
People who were once diagnosed with progressive-relapsing MS (PRMS) are now considered primary progressive.
Other types of MS
The other types of MS are:
- clinically isolated syndrome (CIS)
- relapsing-remitting MS (RRMS)
- secondary progressive MS (SPMS)
These types, also called courses, are defined by how they affect your body.
Each type has different treatments with many therapies overlapping. The severity of their symptoms and long-term outlooks will 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 prognosis of PPM is different for everyone and unpredictable.
Symptoms may become more noticeable over time, especially as you get older and you begin to lose certain functions in organs like your bladder, bowels, and genitals due to age and PPMS.
Here are the main differences between PPMS and SPMS:
- SPMS often begins as a diagnosis of RRMS that eventually becomes more severe over time without any remissions or improvement of symptoms.
- SPMS is always the second stage of an MS diagnosis, while RRMS is an initial diagnosis on its own.
Here are the main differences between PPMS and RRMS:
- RRMS is the most common type of MS (about 85 percent of diagnoses), while PPMS is one of the rarest.
- RRMS is twice to three times as common in women as in men.
- 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 may just have 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, around the 20s and 30s, as opposed to the 40s and 50s with PPMS.
PPMS affects everyone differently.
Common early symptoms of PPMS include weakness in your legs and having trouble walking. These symptoms typically become more noticeable over a period of 2 years.
Other symptoms typical of the condition include:
- stiffness in the legs
- problems with balance
- weakness and tiredness
- trouble with vision
- bladder or bowel dysfunction
- numbness and/or tingling in different parts of the body
The exact cause of PPMS, and MS in general, isn’t known.
The most common theory is that MS begins when your immune system starts attacking your central nervous system. This results in a loss of myelin, the protective covering around nerves in your central nervous system.
While doctors don’t believe that PPMS can be inherited, it may have a genetic component. Some believe that it may be triggered by a virus or by a toxin in the environment that when combined with a genetic predisposition can increase the risk of developing MS.
Work closely with your doctor to help diagnose which of the four types of MS you may have.
Each type of MS has a different outlook and different 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 in order for someone to receive a firm PPMS diagnosis.
Other conditions with symptoms similar to PPMS include:
- an inherited condition that causes stiff, weak legs
- a vitamin B-12 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, your 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 the spinal fluid
- conduct evoked potentials (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
- bladder and bowel problems.
There are many disease-modifying therapies (DMTs) and steroids approved by the FDA for relapsing forms of MS.
These DMTs 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.
One treatment, ibudilast, has been used in Japan for over 20 years to treat asthma and may have some ability to treat inflammation in PPMS.
Another treatment called masitinib has been used for allergies by targeting mast cells involved in allergic reactions and shows promise as a treatment for PPMS, too.
It’s important to note that these two treatments are still very early in development and research.
People with PPMS can relieve symptoms with exercise and stretching to:
- stay as mobile as possible
- limit how much weight you gain
- increase energy levels
Here are some other actions you can take to manage your PPMS symptoms and maintain your quality of life:
- Eat a healthy and nutritious diet.
- Stay on a regular sleep schedule.
- Go to physical or occupational therapy, which can teach you strategies for increasing mobility and managing symptoms.
Four modifiers are used to characterize PPMS over time:
- Active with progression: PPMS with worsening symptoms and relapses or with new MRI activity; increasing disability will also occur
- Active without progression: PPMS with relapses or MRI activity, but no increasing disability
- Not active with progression: PPMS with no relapses or MRI activity, but with increasing disability
- Not active without progression: 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 don’t actually improve. With this form of MS, people don’t regain functions they may have lost.
If you’re living with PPMS, it’s important to find sources of support. There are options to seek support on an individual basis or in the wider MS community.
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 doctor 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 to talk to other people who are living with MS. Consider looking into support groups, either online or in-person.
The National Multiple Sclerosis Society offers a service to help you find local support groups in your area. They also have a peer-to-peer connection program run by trained volunteers who live with MS.
Check in with your doctor regularly if you have PPMS, even if you haven’t had any symptoms for a while and especially when you have more noticeable disruptions in your life by an episode of symptoms.
It’s possible to have a high quality of life with PPMS as long as you work with your doctor to figure out the best treatments as well as lifestyle and dietary changes that work for you.
There’s no cure for PPMS, but treatment makes a difference. Although the condition is progressive, people can experience periods of time where symptoms don’t actively worsen.
If you’re living with PPMS, your doctor will recommend a treatment plan based on your symptoms and general health.
Developing healthy lifestyle habits and staying connected to sources of support can also help you maintain your quality of life and overall well-being.