Primary Progressive Multiple Sclerosis

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Primary Progressive Multiple Sclerosis

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Primary Progressive Multiple sclerosis is a potentially debilitating disease of the brain and spinal cord, in which the immune system attacks the protective sheath of the nerve fibers and disrupts the ability of the brain to communicate with the rest of the body. About 15% of the patients of multiple sclerosis (MS) are diagnosed with primary progressive multiple sclerosis (PPMS). It is one of the four types of multiple sclerosis, and unlike other types of MS, it progresses from the start without relapse or remission. Usually, this medical condition is diagnosed in people in their forties or fifties, though it can be diagnosed earlier or later than this. Equal numbers of men and women suffer with the disease.


Primary progressive multiple sclerosis is caused due to immune system mediated damage of the myelin (protective coating around the nerves of the brain and spinal cord that acts as an insulating sheath). This causes inflammation and progressive damage to the nerves. As a result, the nerve damage disrupts the normal flow of nerve impulses, or the messages from the central nervous system, causing a deterioration or loss of body function. In many cases, the nerve fibers (axons) are completely destroyed later in the course of the disease.

Signs and symptoms:

About 80% patients with primary progressive MS develop problems with their walking. Usually the changes are very subtle and minimal in the beginning, hence go unnoticed, such as mild difficulty running or climbing stairs, or tripping up, particularly if not focused on walking appropriately.

As the condition progresses, patients may find walking to get more difficult. They feel the need of taking rest after walking for a small distance as their legs start to feel weaker.

Usually over a period of many years, this difficulty in walking can lead to progressive stiffness and weakness of both legs. Problems with the legs are common in Primary progressive MS as the disease tends to involve the spinal cord, a vital part of the nervous system for control of movements of legs. Diffuse involvement of spinal cord is precisely the reason for the symptoms of the bladder, bowel and sexual function coexisting with the problems of the legs.

Patients also present with problems of memory or thinking, though usually such symptoms are mild in severity.

Other symptoms include fatigue, numbness, difficulty controlling the bladder or bowels, depression, mood changes, shakiness, sexual dysfunction, vision problems, such as double vision.


The diagnosis of primary progressive MS can take a long time, and there is not a single test that can diagnose it in one go. A number of tests are carried out, in association of the definitive clinical picture indicating to the possibility of primary progressive MS, supportive medical history and findings of physical assessment.

MRI Scan of the brain and spinal cord is advised. Evoked potentials (measuring the speed of electrical activity passing to and from the brain) are measured and a lumbar puncture is carried out to get to the right diagnosis.

Brain MRI has to be interpreted with care because patients with primary progressive MS often have very few lesions in the brain, making it difficult to identify them. Also, such lesions can be visualized in other, non-related neurological conditions.


In March 2017, FDA approved ocrelizumab to treat adult patients with primary progressive MS, making it the first drug approved by the FDA to treat this condition.

Ocrelizumab (Ocrevus) is a monoclonal antibody that acts on the B cells of immune system and totally depletes them from circulation. B cells are involved in causing the damage to the brain and spinal cord tissues in MS and primary progressive MS. Ocrelizumab is recommended to be administered to the patients in the form of intravenous infusion, every six months.

Some other medications can help manage the symptoms of PPMS, such as spasticity, bladder and bowel problems, and sexual dysfunction. These medications include steroids (prednisone) and methotrexate.

High dose biotin, currently undergoing phase 3 trial, has shown promising results, especially with improvement in vision secondary to optic nerve injury.

Besides pharmacological management, many other treatments can alleviate the severity of symptoms of PPMS. These include occupational therapy, physical therapy, speech-language therapy, psychosocial therapy, and exercise.

FDA Approved Medicines