Up to 15 percent of people with MS are diagnosed with primary progressive multiple sclerosis (PPMS).

The disease is named “primary progressive” because it progresses from the onset of the first (primary) symptoms. PPMS symptoms steadily worsen over time without relapses or remissions, unlike relapsing-remitting multiple sclerosis (RRMS), which is characterized by exacerbations followed by periods of symptom-free remission.

The progression of symptoms in PPMS varies between patients. The disease is called “active” when a patient experiences occasional relapses or there is evidence of new lesions on an MRI. “Not active” or “with progression” means there is evidence that a symptom is worsening over time with or without relapse, or new lesions are shown on an MRI.

People with PPMS have fewer brain lesions and inflammation than people with RRMS. Conversely, people with PPMS usually have more spinal cord lesions than people with RRMS.

PPMS diagnosis

Accurate diagnose of PPMS requires at least three characteristics. The patient must have one year of disease progression (worsening of neurological function without remission), and two additional characteristics that could include a brain lesion that is recognized as typical of MS, two or more lesions of a similar pattern in the spinal cord, or evidence of immune system activity in the central nervous system (shown in an elevated IgG index or an oligoclonal band in the spinal fluid).

PPMS diagnosis can take a long time, especially in cases when neurologic symptoms have just begun. In fact, PPMS diagnosis can take two to three years longer than RRMS diagnosis.

Typically, PPMS is diagnosed between 40 and 60 years old, but earlier diagnosis can happen. The disease affects women and men equally.  People with PPMS usually experience more problems with walking and working, and may require assistance for everyday activities.

Treating PPMS

On March 28 2017, Ocrevus (ocrelizumab) became the first treatment approved by the U.S. Food and Drug Administration (FDA) specifically to treat PPMS. In clinical trials, Ocrevus slowed disease progression in PPMS patients.

Other treatments are still in development. There are ongoing clinical trials (such as NCT02284568NCT01776060 and NCT02913157) assessing various experimental PPMS medications.

People who experience relapses or show evidence of disease activity on an MRI are sometimes prescribed approved RRMS disease-modifying therapies that are made to reduce inflammation in the central nervous system.

There are also ways for PPMS patients to manage the disease, beyond medication. Appropriate exercise and healthy eating habits are important for people with MS. When relapse or worsening of symptoms occur, physical and occupational therapy can help minimize muscle weakness and help restore movement.

Be sure to follow the latest developments on PPMS treatments by following Multiple Sclerosis News Today’s Progressive Multiple Sclerosis Experimental Therapy Tracker.

SMA News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

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Dr. Patricia Coyle at CMSC 2016 Discussing Progressive MS Treatment

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