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In primary progressive MS, a rare form of multiple sclerosis, symptoms occur and get progressively worse without the "on-again, off-again" pattern typical of more common types of MS.
Primary progressive multiple sclerosis—PPMS—is a rare form of the disease. The easiest way to understand primary progressive multiple sclerosis is through comparison with the more common forms of MS.
Usually, when someone is diagnosed with MS, they have a form called "relapsing-remitting MS," which has alternating periods with and without symptoms. Over time, the period without symptoms gets shorter until the patient has symptoms most or all of the time. At this point, they move into what is called "secondary progressive MS," which is characterized by persistent symptoms and disability that get worse over time.
PPMS is like secondary progressive MS—constant or frequent symptoms and disability that get gradually worse—but with PPMS, you don't have relapsing-remitting MS first. The main difference between primary and secondary progressive MS is whether the progressive form was the first kind of MS you had (primary) or if it developed after you already had MS (secondary).
Primary progressive MS symptoms are like those of other forms of MS, including:
PPMS takes time to diagnose. At first, your symptoms may be subtle, so it isn't always clear that you have a condition. There's not a single, definitive test for MS. As your symptoms become more noticeable, your doctor will perform tests and discuss your medical history to diagnose primary progressive MS. Common tests include:
Diagnosing PPMS requires symptoms of PPMS for at least a year in combination with either brain scans or spinal tap results consistent with MS.
Medical science doesn't have a good understanding of what causes MS. Your symptoms result from your immune system attacking your nervous system, but doctors don't know exactly why that happens. Some people appear to have a genetic predisposition to autoimmune disorders, which may be part of the reason.
However, genetics appear to play only a minor role in developing MS. Environmental factors may be important since MS rates vary considerably in different parts of the country.
Unfortunately, there aren't many primary progressive multiple sclerosis treatments that address the underlying cause of the disease, so your options are limited compared to treatment for RRMS. Instead, many treatments for primary progressive MS focus on mitigating symptoms and improving or maintaining quality of life.
Learn more about treatment for multiple sclerosis.
Only one drug is currently approved to treat PPMS. It's called ocrelizumab and works by destroying certain cells in your immune system. Apart from ocrelizumab, the best medications for primary progressive MS are specific drugs to treat symptoms such as depression, fatigue, or problems with urination.
Like other forms of MS, primary progressive multiple sclerosis has no cure. However, it's not considered fatal. People with MS live to be almost as old as the general population.
Everyone's experience of PPMS is different. On average, people are about 10 years older when they develop PPMS compared to RRMS, but the disease tends to progress faster and be more debilitating than other forms of MS. However, it can still take years or decades to develop to the point of severe disability.
People with MS live, on average, about six or seven years less than the general population.
PPMS is different than other forms of MS because it usually progresses steadily without the relapse-remission cycle that characterizes RRMS and, to a lesser degree, SPMS.
Experts believe that genetics play only a small role in the development of primary progressive MS.
The difference between primary and secondary progressive MS is that secondary progressive MS develops out of an existing case of relapsing-remitting MS, while PPMS develops independently.
Other differences include:
Many people with primary progressive MS lead long, full lives. However, since the disease is progressive and has no cure, you'll probably have to make accommodations for your condition as you age. For example, you may eventually need a walking aid or a wheelchair to maintain your independence.
Primary progressive MS is rare, occurring in just 10-15% of people diagnosed with MS.
The Multiple Sclerosis Center at Brigham and Women’s Hospital is internationally recognized for providing the most advanced treatment options for people with MS. Its expert care is strengthened by a leading clinical research program at the forefront of discovery.
You can schedule an appointment with our neurology team by calling鈥�617-525-6550鈥痮r by鈥�submitting a request through our secure online form.
What is multiple sclerosis (MS) and what are the symptoms of MS? Learn about testing and treatment options for MS, as well as the best ways to manage symptoms and future inflammatory relapses from Kristin M. Galetta, M.D., Neurologist, Brigham and Women’s Hospital, and Michael Levy, M.D., Ph.D., Neurologist, Massachusetts General Hospital.
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