The Four Courses of MS
People with MS can typically experience one of four disease courses, each of which might be mild, moderate, or severe.
Relapsing-Remitting MS People with this type of MS experience clearly defined attacks of worsening neurologic function. These attacks—which are called relapses, flare-ups, or exacerbations —are followed by partial or complete recovery periods (remissions), during which no disease progression occurs. This remission can last from months to years. Approximately 85% of people are initially diagnosed with relapsing-remitting MS.
Secondary-Progressive MS Following an initial period of relapsing-remitting MS, many people develop a secondary-progressive disease course in which the disease worsens more steadily, with or without occasional flare-ups, minor recoveries (remissions), or plateaus.
Primary-Progressive MS, often called malignant MS in severe cases with rapid progression…this is my MS. Doctors have debated whether I am Primary, or Progressive relapsing…neither are good, and both are termed malignant M.S.
This disease course is characterized by slowly worsening neurologic function from the beginning—with no relapses or remissions. The rate of progression may vary over time, with occasional plateaus, followed by further aggressive attacks and disability. Approximately 10% of people are diagnosed with primary-progressive MS.
Progressive-Relapsing In this relatively rare course of MS (5%), people experience steadily worsening disease from the beginning, but with clear attacks of worsening neurologic function along the way. They may or may not experience some recovery following these relapses, but the disease continues to progress without remissions.
Feeling “left out in the cold” with Malignant MS…this is the MS I live with, struggle with, and fight against every day
My symptoms first began two years ago, and I have gone from zero disability to an EDSS varying from 5.5 – 6 in 24 months, depending on if it is a “good” day or not. Meaning? I use a walker a good deal of the time, and some days I am left fatigued and unable to move, sliding to the ground.
Prognosis? Confined to a wheelchair within 12-18 months, and bed ridden within 24-36 months. If you read over the list of symptoms below, I have every one. Not all at the same time but many, many of them in conjunction with one another – lasting anywhere from 10-24 hours or more. I am never without 3-4 symptoms…never. ALL this could change…with a medical trip to Russia. Nothing has been shown to slow down the progression of primary progressive MS. Nothing…until NOW! Read the above section entitled: Stem Cell Transplants.
“People with primary progressive MS have a different illness — physically and emotionally — than other MS sufferers”, says Rosalind Kalb, PhD, a psychologist with the National MS Society.
“The big thing I see is they feel left out in the cold.” She’s talking about all the advances seen with other forms of MS. “It’s almost like the message is, ‘it’s not for you’ — not the new treatments, the new research, all the clinical trials…not for you. They’re even feeling left out by health care professionals — it’s like ‘we can’t do anything for you.’”
The experience of primary progressive/malignant MS itself is different, too. Unlike people with other forms of MS, who have attacks followed by symptom-free periods, says Kalb, people with a particularly aggressive form of primary progressive MS have, from the beginning, been living with a disease that steadily progresses, day by day, each worse than the day before.
Malignant Multiple Sclerosis
There’s no “bright side” to primary progressive MS. It can strike quite suddenly and severely. The term malignant multiple sclerosis is used to describe MS patients who reach significant level of disability in less than 5 years from their first symptoms. Unfortunately, this is the diagnosis given me by my neurologist – malignant multiple sclerosis. I two years I have gone from skiing to the use of a walker. From and EDSS of 0.0 to 6.0 in less than 24 months. In short…not a good prognosis. Most in this condition
The National MS Society defined malignant multiple sclerosis as: disease with a rapid progressive course, leading to significant disability in multiple neurologic systems or death in a relatively short time after disease onset. Reaching Expanded Disability Status Scale of 6.0 or higher, which is equivalent of needing unilateral support to ambulate (or worse) is generally considered this significant disability level.
What causes primary progressive multiple sclerosis? Just as with multiple sclerosis in general, the cause of PPMS is not known. It is thought that the disease occurs in people who are susceptible to it due to some kind of genetic tendency. It is believed by some to be caused by a trigger, perhaps a virus. A genetic tendency does run in my family, and I was ill with a stubborn virus a few months before symptom onset. In March of 2011, I was skiing blue and black runs, all day, and attacking life with a fervor. Four weeks later, I could barely walk through a door; I walked as if I had been over-served. One of my favorite sayings…”I’m not drunk, I have MS”.
In patients that have these types of symptoms, the MRI scans of their brains show few (if any) lesions and few enhancing lesions. However, an MRI scan of their spines will often show a atrophy, which is a result of axon and oligodenrocyte cell loss and injury.
Tremor and Imbalance (Progressive Cerebellar Syndrome)
A small minority (up to 10%) of people with PPMS have these types of symptoms, which are characterized by:
While rare, the following symptoms can also be signs of PPMS:
Why won’t most insurance companies pay for a disease modifying therapy for PPMS?
People who are diagnosed with PPMS are understandably disappointed and frustrated by the unwillingness of insurance companies to cover the costs of medication that other people with MS are taking. While it may not solve the problem of how unfair it feels, it will help them to understand that the insurance industry relies on certain facts – a drug’s cost, data from pivotal trials, and FDA recommendations – to decide which medications to cover for which patients. The unfortunate fact that the existing medications do not seem to be effective in PPMS is sufficient reason for insurers to decide not to cover them for this disease course.
Ebers, George C. Natural history of primary progressive multiple sclerosis. Multiple Sclerosis. 2004; 10: S8-S15.
Thompson, Alan. Overview of primary progressive multiple sclerosis (PPMS): similarities and differences from other forms of MS, diagnostic criteria, pros and cons of progressive diagnosis. Multiple Sclerosis. 2004; 10: S2-S7.
Miller DH, Leary SM. Primary-progressive multiple sclerosis. Lancet Neurol. 2007 Oct;6(10):903-12.
Courtney, Susan Wells. Primary-Progressive Multiple Sclerosis: The Less Talked-about Form of MS. The Motivator. (Published by the Multiple Sclerosis Association of America) Winter/Spring 2007; 44-47, 63.
National Multiple Sclerosis Society
The Cleveland Clinic