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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.

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 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. The rarest form, primary progressive multiple sclerosis, is progressive from the start. There are no acute relapses or remissions in PPMS. About 15 percent of people with MS are diagnosed with PPMS.

Symptoms

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 have difficulty walking. Other symptoms include:

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

Diagnosis

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.

PPMS progresses once symptoms start to present, hence the name primary progressive. There may be periods of active progression and then periods of inactive progression of symptoms and disability. What differentiates PPMS from the relapsing forms is that while active progression may stop temporarily, the symptoms do not resolve. In relapsing forms, the symptoms may actually improve or return near to where they were before the most recent relapse. Another difference is that there’s not as much inflammation in PPMS compared to relapsing forms. Because of this, many of the drugs that work for relapsing forms do not work for PPMS or SPMS. Also, with PPMS, there are fewer lesions in the brain and more in the spinal cord. The progression of symptoms can worsen over a few months or several years.

Causes

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 relapsing forms of MS are more common in women than in men, both sexes get PPMS at about the same rate. PPMS tends to occur about 10 years later in life than the relapsing forms of MS.

Treatment

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 is one medication called ocrelizumab that is approved for PPMS as well as relapsing forms of MS. Immunosuppressants are commonly used in relapsing forms because they decrease inflammation. Since PPMS does not involve significant inflammation, these drugs may not be recommended. In addition to treating the disease, treatment focuses on relieving symptoms.

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.

Outlook

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.

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