Primary progressive multiple sclerosis (PPMS) is one of the four types of multiple sclerosis (MS).
According to the National Multiple Sclerosis Society, about 15 percent of people with MS receive a diagnosis of PPMS.
Unlike other types of MS, PPMS progresses from the start without acute relapses or remissions. Although the disease usually progresses slowly and may take years to diagnose, it typically leads to problems with walking.
There’s no known cause of MS. However, many treatments can help prevent the progression of PPMS symptoms.
Most existing MS drugs are designed to control inflammation and reduce the number of relapses.
However, PPMS causes significantly less inflammation than relapsing-remitting multiple sclerosis (RRMS), the most common type of MS.
In addition, although there might be occasional small degrees of improvement, PPMS doesn’t have remissions.
Because it’s impossible to predict the course of PPMS progression in any individual who has it, it’s difficult for researchers to evaluate the effectiveness of a drug on the course of the disease. However, as of 2017, one PPMS drug has received approval from the Food and Drug Administration (FDA).
Ocrelizumab (Ocrevus) is FDA-approved to treat both PPMS and RRMS.
It’s a monoclonal antibody that destroys certain B cells of the immune system. Research suggests that B cells are partially responsible for damage to the brain and spinal cord tissues of people with MS. This damage is enabled by the immune system itself.
Ocrelizumab is administered by intravenous infusion. The first two infusions are administered 2 weeks apart. Later infusions are administered every 6 months.
The goal of using stem cells to treat PPMS is to promote the immune system to repair damage and reduce inflammation in the central nervous system (CNS).
For a process known as hematopoietic stem cell transplantation (HSCT), stem cells are collected from a person’s own tissues, like bone marrow or blood, and then reintroduced after their immune system has been suppressed. This is done in a hospital setting and is currently FDA approved.
However, HSCT is a major procedure with serious side effects. More research and outcomes from clinical trials are needed before this becomes a widely used treatment for PPMS.
Several clinical trials are currently underway in people with PPMS. Clinical trials go through several phases before they receive FDA approval.
Phase I focuses on how safe the drug is and involves a small group of participants.
During phase II, researchers aim to determine how effective the drug is for certain conditions like MS.
Phase III typically includes a larger group of participants.
Researchers also look at other populations, dosages, and drug combinations to find out more about how safe and effective the drug is.
A two-year phase II study is currently evaluating the oral antioxidant lipoic acid. Researchers are studying whether it can preserve mobility and protect the brain more than an inactive placebo in progressive forms of MS.
This study builds on an earlier phase II study that looked at 51 people with secondary progressive MS (SPMS). Researchers found that lipoic acid was able to reduce the rate of brain tissue loss compared to placebo.
Biotin is a component of the vitamin B complex and is involved in cell growth and the metabolism of fats and amino acids.
An observational study is recruiting people with PPMS who are taking a high dose of biotin (300 milligrams) daily. Researchers want to see if it’s effective and safe in slowing the progression of disability in people with PPMS. In observational studies, researchers monitor participants without intervening in the process.
Another phase III study is evaluating a high-dose biotin formulation known as MD1003 to see if it’s more effective than a placebo. Researchers want to know if it can slow the disability of people with progressive MS, especially those with gait impairment.
A small open-label trial looked at the effects of high-dose biotin in people with either PPMS or SPMS. Doses ranged from 100 to 300 milligrams per day for 2 to 36 months.
Participants in this trial showed improvement in visual impairment related to optic nerve injury and other MS symptoms, such as motor function and fatigue.
However, another study found that high-dose biotin nearly tripled the relapse rate in participants with PPMS.
The FDA has also warned that high doses of biotin may lead to inaccurate lab results for people with certain conditions, including MS.
Masitinib is an oral immunomodulatory drug that has been developed as a possible treatment for PPMS.
Ibudilast inhibits an enzyme called phosphodiesterase. Used as an asthma medication mainly in Asia, it has also been shown to promote myelin repair and help protect nerve cells from damage.
Ibudilast was awarded fast track designation by the FDA. This could speed its future development as a possible treatment for progressive MS.
Results of a phase II trial in 255 patients with progressive MS were published in The New England Journal of Medicine.
In the study, ibudilast was associated with slower progression of brain atrophy than a placebo. However, it also led to higher rates of digestive system side effects, headache, and depression.
Many other treatments, aside from medications, can help optimize functioning and quality of life despite the effects of the disease.
Occupational therapy teaches people the practical skills they need to take care of themselves both at home and at work.
Occupational therapists show people how to preserve their energy, since PPMS typically causes extreme fatigue. They also help people adjust their daily activities and chores.
Therapists may suggest ways to improve or renovate homes and workplaces to make them more accessible to people with disabilities. They may also assist in treating memory and cognitive problems.
Physical therapists work to create specific exercise routines to help people increase their range of motion, preserve their mobility, and reduce spasticity and tremors.
Physical therapists can recommend equipment to help people with PPMS get around better, such as:
Speech-language pathology (SLP)
Some people with PPMS have problems with their language, speech, or swallowing. Pathologists can teach people how to:
- prepare food that’s easy to swallow
- eat safely
- use feeding tubes properly
They may also recommend useful telephone aids and speech amplifiers to make communicating easier.
Exercise routines can help you reduce spasticity and maintain a range of motion. You can try yoga, swimming, stretching, and other acceptable forms of exercise.
Of course, it’s always a good idea to discuss any new exercise routine with your doctor.
Complementary and alternative (CAM) therapies
CAM therapies are considered nonconventional treatments. Many people incorporate some type of CAM therapy as part of their MS management.
There is very limited research evaluating the safety and effectiveness of CAM in MS. But such therapies are intended to help prevent the disease from damaging your nervous system and maintain your health so your body won’t feel so much of the effects of the disease.
According to one study, the most promising CAM therapies for MS include:
- a low-fat diet
- omega-3 fatty acid supplements
- lipoic acid supplements
- vitamin D supplements
Talk to your doctor before adding CAM to your treatment plan, and make sure you continue to adhere to your prescribed treatments.
Common MS symptoms you may experience include:
- cognitive impairment
- urinary problems
- mood changes
A big part of your overall treatment plan will be to manage your symptoms. You may need a variety of medications, lifestyle changes, and complementary treatments to do this.
Depending on your symptoms, a doctor may prescribe:
- muscle relaxants
- medications for bladder dysfunction
- medications to reduce fatigue, such as modafinil (Provigil)
- pain medications
- sleeping aids to help with insomnia
- medications to help treat erectile dysfunction (ED)
These lifestyle changes could make your symptoms more manageable:
- Eat a healthy diet rich in vitamins, minerals, and antioxidants.
- Perform strength-building exercises to build up muscles and boost energy.
- Try gentle exercise and stretching programs like tai chi and yoga to help with balance, flexibility, and coordination.
- Maintain a proper sleep routine.
- Manage stress with massage, meditation, or acupuncture.
- Use assistive devices to improve quality of life.
The goal of rehabilitation is to improve and maintain function and reduce fatigue. This may include:
- physical therapy
- occupational therapy
- cognitive rehabilitation
- speech-language pathology
- vocational rehabilitation
Ask your doctor for a referral to specialists in these areas.
PPMS isn’t a common type of MS, but multiple researchers are still exploring ways to treat the condition.
The 2017 approval of ocrelizumab marked a big step forward because it is approved for PPMS indication. Other emerging treatments, such as anti-inflammatories and biotin, have garnered mixed results in PPMS so far.
Ibudilast has also been studied for its effects on PPMS and SPMS. Recent results from a phase II trial show that it causes some side effects, including depression. However, it was also associated with a lower rate of brain atrophy.
Speak with your doctor if you want the most up-to-date information on the best ways to manage your PPMS.