Progressive Relapsing Multiple Sclerosis

Progressive relapsing multiple sclerosis is the rarest form of MS, affecting only about 5% of people with multiple sclerosis. Unlike other types, this condition combines steady worsening of symptoms from the start with occasional sudden flare-ups, making it particularly challenging to manage.

Table of contents

• What Is Progressive Relapsing Multiple Sclerosis?
• Parts of the Body Affected
• Symptoms
• Causes and Risk Factors
• How Is PRMS Diagnosed?
• Treatment Approaches

What Is Progressive Relapsing Multiple Sclerosis?

Progressive relapsing multiple sclerosis (PRMS) is the least common form of multiple sclerosis, a disease that affects the central nervous system, which includes the brain, spinal cord, and optic nerve^[1]. Multiple sclerosis causes destruction of myelin, the protective coating that surrounds and protects nerve fibers (also called axons). This damage disrupts the normal flow of messages from the central nervous system, causing a reduction or loss of body function. In many cases, the nerve fibers themselves are also destroyed^[1].

What makes PRMS different from other types of multiple sclerosis is its pattern of progression. The condition is characterized by a progressive worsening from the beginning, similar to primary-progressive multiple sclerosis. However, people with PRMS also experience occasional relapse episodes where symptoms suddenly intensify, similar to those experienced by people with relapsing-remitting MS^[1].

The progressive worsening in PRMS is caused by nerve damage or loss, while the relapse episodes are due to inflammation^[1]. There are three major types of MS: relapsing-remitting, primary progressive, and progressing relapsing^[2].

Parts of the Body Affected

• Brain
• Spinal cord
• Optic nerve

Symptoms

Symptoms of progressive relapsing multiple sclerosis are similar to those of the primary-progressive variant of the disease. The main symptom of both forms is a gradual worsening of disability^[1].

This gradual worsening may be experienced through increased:

• Fatigue
• Numbness or tingling
• Vision problems, such as double vision
• Spasticity or stiffness of the muscles
• Difficulty controlling the bladder or bowels
• Problems with cognition, such as learning and memory or information processing
• Difficulty with walking and coordination
• Muscle weakness
• Paralysis
• Dizziness
• Shakiness
• Mood changes
• Depression
• Sexual dysfunction

A relapse (also called an exacerbation or attack) is when symptoms suddenly appear or become significantly worse for a period of time. During a relapse, people with MS will have a temporary worsening or recurrence of existing symptoms or the appearance of new symptoms. This typically lasts for a few days to a few months^[1].

Causes and Risk Factors

Only 5 percent of all multiple sclerosis patients are diagnosed with PRMS, and its cause is unknown^[1].

Since the risk of multiple sclerosis is significantly higher when a parent has been diagnosed with the disease, genetic factors may play a role. The unusual relationship between a person’s geographic location during childhood and the risk of MS later in life suggests there may be environmental factors at work in the disease^[1].

Patients with PRMS tend to be diagnosed in their mid-to-late 30s, and the condition affects men and women equally^[1].

How Is PRMS Diagnosed?

Diagnosis begins with a detailed medical history and neurological examination. Because multiple sclerosis can have symptoms similar to those of other nervous system disorders, diagnostic tests will help rule out other causes and confirm a diagnosis^[1].

These tests may include:

• A magnetic resonance imaging (MRI) scan of the brain and spinal cord
• A lumbar puncture, also known as a spinal tap, and cerebrospinal fluid analysis
• Nerve function tests
• Blood tests to rule out other conditions that have similar symptoms

PRMS is often initially diagnosed as primary progressive MS because both conditions share the characteristic of steady progression from the beginning^[1].

Treatment Approaches

Treatment of multiple sclerosis has two main aspects: therapy for the underlying immune disorder and therapies to relieve or modify symptoms^[9].

When treatment is necessary, relapses are usually treated with a high-dose course of powerful corticosteroids (a type of steroid) over a period of three to five days. These are given by intravenous (IV) infusion, providing the drug directly into the bloodstream for a quicker response. As an alternative to IV steroids, treatment for a moderate to severe relapse also includes an equivalent, high-dose, oral regimen of steroids^[8].

Corticosteroids work by reducing inflammation in the central nervous system. While they usually lessen the severity and duration of a relapse, they do not appear to affect the long-term progression of the disease^[8].

To treat relapses, physicians may prescribe corticosteroids such as methylprednisolone or dexamethasone, both given by IV infusion, or via an equivalent, high-dose, oral regimen of these steroids. An oral corticosteroid, prednisone, may be prescribed after the high-dose treatment to ease the patient off the treatment, tapered over one to two weeks^[8].