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Primary-Progressive MS (PPMS): Symptoms and Diagnosis

Medically reviewed by Deborah Weatherspoon, PhD, RN, CRNA on May 19, 2017Written by Renata Ilitsky on April 26, 2016

What is PPMS?

Multiple sclerosis (MS) is the most common disease of the central nervous system. It’s caused by an immune response that destroys the myelin sheath, or coating on nerves.

Primary progressive multiple sclerosis (PPMS) is one of the four types of MS. The three other types of MS are:

PPMS is one of the least common types, affecting about 10 percent of all people diagnosed with MS.

How is PPMS different from other types of MS?

Most people affected by MS have acute attacks with symptoms, called relapses, and periods of months or years with little to no symptoms, called remissions.

PPMS is different. The disease progresses once symptoms start to appear, hence the name primary progressive. There may be periods of active progression and then periods of inactive progression of symptoms and disability.

One difference between PPMS and the relapsing forms is that while active progression may stop temporarily, the symptoms don’t resolve. In relapsing forms, the symptoms may actually improve or return near where they were before the most recent relapse.

Another difference is that there isn’t as much inflammation in PPMS compared to relapsing forms. Because of this, many of the drugs that work for relapsing forms don’t work for PPMS or SPMS. The progression of symptoms can worsen over a few months or several years.

PPMS is often diagnosed in people in their 40s and 50s. RRMS, on the other hand, usually presents in people in their 20s and 30s. PPMS also affects both sexes equally, while RRMS affects two to three times as many women as men.

What causes PPMS?

PPMS is caused by slow nerve damage that stops nerves from sending signals to each other. All four types of MS involve damage to the protective coating (myelin) of the central nervous system, called demyelination, as well as damage to the nerve.

What are the symptoms of PPMS?

PPMS symptoms are similar to SPMS symptoms. Of course, what one person experiences will be different from another.

Symptoms of PPMS may include the following:

Muscle spasticity

A continuous contraction of certain muscles may cause stiffness and tightness, which may affect movement. This can make it more difficult to walk, use the stairs, and influence your overall activity level.

Fatigue

About 80 percent of those with PPMS experience fatigue. This can significantly affect daily life and make it difficult to work and complete regular activities. Those diagnosed with PPMS may find themselves very tired from simple activities. For example, the task of cooking dinner could wear them out and require them to take a nap.

Numbness/tingling

Another early symptom of PPMS is numbness or tingling in various body parts, such as your face, hands, and feet. This can be confined to one area of your body, or travel to other parts.

Problems with vision

This can include double vision, blurred vision, inability to identify colors and contrasts, and pain when moving your eyes.

Issues with cognition

While PPMS typically affects mobility, some individuals may experience a cognitive decline. This can significantly impair remembering and processing information, solving problems, focusing, and learning anything new.

Dizziness

Those with PPMS may have episodes of dizziness and lightheadedness. Others may experience vertigo, a sensation that they’re spinning and losing their balance.

Bladder and bowel problems

Bladder and bowel problems can range from incontinence, to the constant need to go, to constipation. This can lead to sexual problems, such as decreased sex drive, difficulty maintaining an erection, and less sensation in the genitals.

Depression

About half of all people with MS will face at least one depressive episode. Although it’s common to be upset or angry about the increasing disability, these mood changes typically go away with time. Clinical depression, on the other hand, doesn’t subside and requires treatment.

How is PPMS diagnosed?

PPMS has similar symptoms to other types of MS, as well as other nervous system disorders. As a result, it may take up to three years longer to get a confirmed PPMS diagnosis than a RRMS diagnosis.

To get a confirmed PPMS diagnosis, you must:

  • have a year of progressively worsening neurologic function
  • meet two of the following criteria:
    • a brain lesion common to MS
    • two or more similar lesions in your spinal cord
    • presence of proteins called immunoglobulins

Your doctor will likely conduct a medical history exam and ask you about any previous neurologic events. They may ask for family members to be present, as they can contribute their experiences with past symptoms. Your doctor will then likely do a thorough physical exam, specifically checking your nerves and muscles.

Your doctor will order an MRI scan to check for lesions in the brain and spinal cord. They may also order an evoked potentials (EP) test to check for electrical activity in the brain. Finally, your doctor will perform a spinal tap to look for signs of MS in the spinal fluid.

Treatment options

There’s no cure for PPMS. One medication, ocrelizumab (Ocrevus), is approved for PPMS as well as relapsing forms of MS. Immunosuppressants are commonly used in relapsing forms, however, because they decrease inflammation. PPMS doesn’t have a lot of inflammation, so immunosuppressants may not be recommended as useful. Research on effective treatments is ongoing.

Outlook

While there’s no cure for PPMS, those diagnosed with PPMS shouldn’t give up hope. With the help of doctors, physical therapy professionals, speech pathologists, and specialists in mental health, there are ways to manage the disease. These can include medications that help relieve symptoms, such as muscle relaxants for muscle spasms, as well as a healthy diet, exercise, and a proper sleep routine.

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