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What Is Primary-Progressive MS?

The four basic forms of MS

Multiple sclerosis (MS) is a chronic disease that affects the central nervous system. Nerves are coated in a protective layer called myelin, which speeds up the transmission of nerve signals. People with MS experience progressive deterioration of myelin.

Did you know?
Clinically isolated syndrome (CIS) is a newly defined type of MS. People who were previously diagnosed with progressive-relapsing MS (PRMS) are now considered primary progressive: active or not active.

The four main types of MS are:

  • clinically isolated syndrome (CIS)
  • relapsing-remitting MS (RRMS)
  • primary-progressive MS (PPMS)
  • secondary-progressive MS (SPMS)

Relapsing-remitting MS (RRMS) is the most common form of the disease. People with RRMS have acute attacks followed by periods of remission in which the disease doesn’t progress. When this form becomes progressive, it’s called secondary-progressive MS (SPMS). The rarest form, primary-progressive multiple sclerosis (PPMS), is progressive from the start. There are no acute relapses or remissions in PPMS. About 10 percent of people with MS are diagnosed with PPMS.


People with PPMS experience a steady deterioration of neurologic function. It becomes increasingly difficult for signals from the brain to get through to the rest of the body. There are no clear relapses and remissions like that of RRMS. However, the rate of progression can vary over time.

Symptoms include numbness, weakness, and dizziness. People with PPMS may have trouble with balance and coordination, or difficulty walking. Other symptoms include:

  • visual disturbances
  • bladder dysfunction
  • sexual dysfunction
  • cognitive changes or mood disorders, including depression


Diagnosing any form of MS can take a long time. That’s because no single test can confirm MS. The process begins with a detailed patient history and a complete neurological examination. To diagnose MS, your doctor may perform a spinal tap, visual tests, and evoked potential (EP) tests. EP tests measure the brain’s response to electrical stimulation.

An MRI scan of the brain and spinal cord can also help doctors identify lesions, a telltale sign of MS. Before making the diagnosis, other conditions with similar symptoms must be ruled out. Once the diagnosis is made, progression from the start and lack of clear relapses indicate a progressive course.


The exact cause of MS is unknown, but doctors have suggested two different theories. One is that MS begins as an inflammatory process of the autoimmune system that causes loss of myelin. The second is that the disease may be an immune response triggered by a viral infection. This can later cause nerve degeneration or damage.

Research into what may trigger the immune system response responsible for MS is ongoing. Possibilities include viruses or environmental toxins. Why some people develop PPMS is also unknown. MS in any form isn’t contagious.

Who gets PPMS?

MS is more common in Caucasians than in other ethnic groups. Although MS is more common in women than in men, both sexes get PPMS at about the same rate. Most people with PPMS begin to notice symptoms while in their 30s.


Currently, there’s no cure for MS. While many long-term therapies are approved for use in RRMS, they’re not effective in slowing progression in people with PPMS. There are no disease-modifying medications to treat PPMS. Treatment is focused on relieving symptoms. Immunosuppressive therapies have shown positive effects in helping those with progressive forms of MS. Still, this is only a temporary solution or remedy.

Targeted therapies may help symptoms such as bladder and bowel dysfunction, erectile dysfunction, and spasticity. Over-the-counter and prescription medications can help relieve aches and pains. Your treatment depends entirely upon your symptoms and your overall health.

Maintaining your quality of life

Taking care of your overall health can improve your quality of life. Here are some basic tips:

  • Choose a diet rich in nutrients and low in empty calories.
  • Get regular exercise to help strengthen muscles and boost energy.
  • Practice gentle exercise programs like tai chi and yoga to help with balance, flexibility, and coordination.

Some people find that massage, mediation, or acupuncture help to relieve stress and ease pain. Occupational therapy can also be beneficial. Take advantage of assistive devices that can help you function and maintain your independence. Don’t hesitate to apply for a special parking permit.


As with other forms of MS, symptoms vary tremendously from person to person, as does the rate of progression. Over time, people with PPMS may develop problems walking and moving around independently.

Fatigue and other symptoms may make it difficult to perform everyday activities. However, most people with MS have a normal or near-normal lifespan. If you have PPMS, be sure to see your doctor on a regular basis.