Up to 85% of the people who receive a diagnosis of the neurodegenerative disorder multiple sclerosis (MS) have the relapsing-remitting (RR) disease course. This means that people have relapses -- also called attacks or exacerbations -- with new or increasing central nervous system (CNS) symptoms lasting more than 24 hours in the absence of fever or infection.
These attacks are followed by periods of partial or even complete recovery (remissions), where symptoms may disappear, or some may continue and become permanent. In RRMS, the disease does not show any signs of progression during the remission periods. Even though an individual is not experiencing symptoms, however, it does not mean the disease is not present.
Individuals who have progressive, gradual onset of neurologic symptoms over time have PPMS as compared with those who have their first neurologic symptom in the form of a relapse. In PPMS, the symptoms typically worsen steadily over time and physical and/or cognitive disability accumulates. This represents about 10-15% of people with MS.
Patients are often understandably distressed when they learn they have PPMS, noted Sammita Satyanarayan, MD, of the Icahn School of Medicine at Mount Sinai in New York City.
"It's easy for patients to imagine that their loss of function will keep going at the same rate and that they will end up in a wheelchair in a set period of time, but it's often not like that," she told ľֱ. "Instead, there might be periods where symptoms stabilize -- sometimes for a long time -- before worsening again."
Diagnosing PPMS
MS can be challenging for doctors to diagnose because there is no single test that identifies it. Your doctor will rely on a variety of tests to make the diagnosis, including physical and neurological exams, magnetic resonance imaging (MRI), and spinal fluid analysis. One of the most important tools to the diagnosis is listening to your story of your symptoms by taking a clinical history.
For your doctor to make a diagnosis of RRMS, you must have at least two attacks of neurological symptoms at least 1 month apart, and the MRI must show evidence of scarring in the brain and/or spinal cord.
PPMS takes a bit longer to diagnose. That's because you must have 1 year of disease progression (worsening of neurological function without remission), along with two of the following:
- A specific type of lesion in the brain that is typical of MS
- At least two lesions of a similar type in the spinal cord
- Evidence in the spinal fluid that shows immune system activity in the central nervous system
Studies have shown that primary progressive MS may take 2-3 years longer to diagnose than relapsing-remitting MS and people with PPMS are on average about 10 years older at diagnosis compared with those with other forms of the disease.
PPMS affects the spinal cord more than the brain, so people with this form often have more problems walking and may find it challenging to continue working. The National MS Society has that can help.
Secondary Progressive MS
Many people who receive an initial diagnosis of relapsing-remitting MS gradually worsen over time, separate from exacerbations. This transition is called secondary progressive (SP) MS, in which steady disease progression often replaces the relapse/remission cycle.
Early aggressive treatment shows promise as a way to improve long-term outcomes for patients with relapsing forms of MS, including SPMS. Neurological damage begins in the early stages of MS, sometimes even before the first symptoms develop, so many doctors believe that it's better to start with more effective treatments as soon as possible.
Recent studies have shown that disease-modifying treatments (DMTs) can have a positive long-term impact on patients when they start taking the medications soon after diagnosis instead of waiting for the disease to worsen.
Treating Progressive MS
The early aggressive treatment approach, however, does not seem to work as well in patients with PPMS. That's because the primary progressive symptoms tend to be caused by neurons in the CNS physically breaking down, as opposed to the inflammation seen in relapsing MS.
Currently, ocrelizumab is the only FDA-approved DMT for PPMS. Compared with placebo, ocrelizumab delayed disability progression in PPMS in a large clinical trial.
Patients receive ocrelizumab intravenously in their doctor's office every 6 months. Patients with hepatitis B infections should not use ocrelizumab. Common side effects include upper and lower respiratory tract infections and skin infections.
Ocrelizumab has FDA approval for use in patients with SPMS, although those with SPMS also have many other treatment options, including:
- Cladribine (Mavenclad), an oral therapy approved in 2019
- Ofatumumab (Kesimpta), a self-injection therapy approved in 2020
- Siponimod (Mayzent), an oral therapy approved in 2019
- Ublituximab-xiiy (Briumvi), an IV therapy approved in 2022
Types of SPMS
There are four types of secondary progressive MS: active, active-progressing, non-active progressing, and stable. Doctors treat each one differently.
- In active SPMS, you still have relapses, which means you might be able to take the same DMT you did when the disease was relapsing-remitting.
- In active progressing SPMS, you still have relapses, but your symptoms gradually get worse. You may need a stronger DMT at this point.
- In non-active progressing MS, you no longer have relapses, but your symptoms are getting worse.
- In stable SPMS, the disease is not progressing.
At any point, treatment may focus on symptoms and other common issues in MS, including pain, depression, and challenging sleep patterns.
Rehabilitation Therapies Can Help
Regardless of the type of MS you have, your doctor may prescribe various rehabilitation therapies to help you increase strength and preserve function. These therapies include:
- Physical therapy, which focuses on strength and ease of movement. Your physical therapist can help you learn to use mobility aids like a cane or a scooter
- Occupational therapy, which focuses on activities of daily living like dressing and cooking, and making sure your home setup is effective
- Cognitive rehabilitation – MS can cause problems with thinking and memory, and this type of therapy aims to help you manage such problems
- Speech-language therapy – A speech-language therapist will help you speak clearly and eat and swallow safely
Always keep your doctor informed about what your medical goals are. They will suggest treatments that mesh with those goals at all stages of the disease. Together, you will make decisions about maximizing function and minimizing MS symptoms while keeping your quality of life high.
Read previous installments in this series:
For Your Patients: What to Expect After a Multiple Sclerosis Diagnosis
For Your Patients: What Are the Different Types of MS?
For Your Patients: Coping With the Mental Health Challenges of Multiple Sclerosis
For Your Patients: The Benefits of Early Aggressive Treatment for MS
"Medical Journeys" is a set of clinical resources reviewed by doctors, meant for physicians and other healthcare professionals as well as the patients they serve. Each episode of this journey through a disease state contains both a physician guide and a downloadable/printable patient resource. "Medical Journeys" chart a path each step of the way for physicians and patients and provide continual resources and support, as the caregiver team navigates the course of a disease.