10 Essential Facts About Primary-Progressive MS
With only one FDA-approved drug available for PPMS, this type of multiple sclerosis remains difficult to treat, but adaptive devices and wellness programs can help you manage your symptoms.
For many people with multiple sclerosis (MS), treatment is focused on preventing and managing relapses — acute symptoms that are often absent for long stretches of time. Ultimately the disease can result in disability in walking, thinking, and working.
Despite the challenges that PPMS presents, great strides have been made in its treatment and management. Here are 10 things you should know about PPMS.
1. There’s Often No Easily Identifiable Pattern of Symptoms in PPMS?
While relapsing forms of MS tend to cause an?acute neurological episode that’s hard to ignore, the onset of PPMS “is much more gradual and insidious,” according to Patricia K. Coyle, MD, a neurologist and the director of the multiple sclerosis comprehensive care center at Stony Brook University School of Medicine in Stony Brook, New York.
Classic symptoms of PPMS, according to Dr. Coyle, include difficulty walking, leg weakness, and muscle spasticity. But because the onset of these symptoms is gradual, “People are more apt to write it off as, ‘I’m getting older, I’m getting clumsier,’” she says. “I think it’s very easy to miss it for a period of time.”
- Pain, including headaches
- Numbness
- Lhermitte sign, or electric-like impulses that shoot down your back and legs when you bend your neck
- Vision issues
- Difficulty with balance
- Dizziness
- Depression or mood changes
- Bladder and bowel control problems
- Sexual issues
2. It’s Unclear Exactly What Causes PPMS
According to Coyle, PPMS is believed to be the result of injury to the myelin that surrounds axons in the brain and spinal cord, as well as to the axons themselves. This neurodegeneration tends to affect all?people with MS over time, but it’s unknown why people with PPMS rarely experience the focal inflammation that causes acute symptoms typical of people with relapsing forms of MS.
“Whether there’s a fundamental difference between primary-progressive MS and relapsing MS is not clear,” says Coyle.
There are certain genetic and environmental factors associated with the risk of MS, but none of them have been shown to predict the type of MS someone develops.
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3. PPMS Affects Only About 15 Percent of People With MS
4. The Onset of PPMS Tends to Occur Later Than Relapsing Forms of MS
While the reason for this later onset isn’t fully understood, Coyle says it may reflect neurodegeneration (degeneration of the nervous system) that shows up clinically at midlife, when there has been a certain amount of loss of central nervous system function.
5. People With PPMS Can Stabilize for Up to Several Years
In some people with PPMS, Coyle says, the gradual worsening of symptoms that characterizes this form of the disease will abate for up to a few years, for unknown reasons. Sometimes, she says, “You can even see little blips of improvement,” but inevitably this improvement is reversed, and a person’s neurological deficit worsens.
Even when a person with PPMS appears to be clinically stable in terms of symptoms, Coyle says,?MRI scans typically show continuing damage to their central nervous system.
6. There Is Now an FDA-Approved Drug to Treat PPMS
Ocrevus was initially available only as an intravenous (IV) infusion, typically given about every six months after the first infusion, which is administered as two separate infusions two weeks apart. However, in September 2024 the FDA?approved a subcutaneous version of ocrelizumab called Ocrevus Zunovo. The twice-a-year, under-the-skin dosing regimen gives people living with MS another treatment delivery option. Ocrevus also is approved for relapsing forms of MS.
7. Ocrevus May Not Work Well in Everyone With PPMS
Taking Ocrevus if you’re older than 55 can come with risks, adds Coyle, who says Ocrevus is known to suppress the immune system. Those who take it are often more vulnerable to infections, which could pose additional risks in older people. “We know that as the body ages, the immune system ages, and you’re more likely to see things go awry,” she explains.
8. Even With Treatment, PPMS Leads to Gradual Loss of Neurological Function
Progressive forms of MS, including PPMS, are considered more severe than relapsing-remitting MS because they inevitably lead to disability, according to Coyle. “Once a patient enters or is in a progressive stage,” she says, “there is going to be gradual deterioration.”
9. It’s Important to Treat Symptoms of PPMS, Not Just the Disease Process
While there’s only one disease-modifying treatment with a modest benefit for PPMS, Coyle emphasizes that neurologists can still do a lot to help people with the condition manage their symptoms and improve their quality of life.
This means, according to Coyle, treating any spasticity, cramps, or pain, and talking about adaptive devices and behaviors to make daily tasks less arduous.
One type of adaptive device Coyle recommends is an all-terrain mobility chair, which can allow you to maneuver over rocky terrain, go up and down steps, or travel in other areas that might not be suitable for a normal wheelchair.
Another option is a smart-watch alert system. “For people with fairly significant gait impairment, this could actually notify people about a fall. They may be able to press an alert button. You could even have some smart-watch apps that will call in or check on individuals,” she explains.
10. Attention to Overall Wellness May Help Slow the Progression of PPMS
Coyle notes that in recent years it’s become increasingly clear how important a general wellness program is for people with PPMS. She emphasizes how important it is not to smoke, to maintain a healthy body weight, to do regular exercise, and to make sure you’re not deficient in vitamin D.
The Takeaway
Primary-progressive MS, which affects up to 15 percent of people with MS, can be more difficult to diagnose and to treat than the relapsing form of the condition. While it can be challenging to manage, addressing the symptoms and maintaining your overall health may help slow the progression.
Additional reporting by Christina Vogt.
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Jason Paul Chua, MD, PhD
Medical Reviewer
Quinn Phillips
Author
A freelance health writer and editor based in Wisconsin, Quinn Phillips has a degree in government from Harvard University. He writes on a variety of topics, but is especially interested in the intersection of health and public policy. Phillips has written for various publications and websites, such as Diabetes Self-Management, Practical Diabetology, and Gluten-Free Living, among others.