Let’s continue looking at characteristics of Progressive MS. I already talked about the different types of MS and focused specifically on Secondary Progressive. As this series continues, I will discuss research and clinical trials, medications, “a day in the life,” and other topics relating to the progressive types of MS. Today the focus is on Primary Progressive and Progressive/Relapsing MS.
Remember, most MSers have Relapsing/Remitting MS (55-85%). Only a small minority of MSers have Primary Progressive (10-15%) and Progressive/Relapsing MS (2-5%). There is an information void for these types. That information can be confusing to the patients, and maybe even to the doctors who see so few cases compared to the other types.
All types of progressive MS are characterized by a gradual increase in loss of function leading to disability. The types of progressive MS differ only in disease course and timing. Notice how small the PP and PR boxes (in blue) are on the graphic.
Primary Progressive MS (PPMS)
If the initial MS diagnosis is not Relapsing/Remitting MS, it is usually Primary Progressive. Somewhere between 10% and 15% of all MSers have this type.
From the beginning, PPMS is a steady progression of greater affliction with little or no recovery. It is usually diagnosed about one or two years after the initial presentation of symptoms, based on:
- a neurological history
- a primary MRI, or a series of spinal MRIs
- evoked potentials tests indicating a slowing in nerve conduction speeds, especially a higher incidence of delayed visual evoked potentials
- a spinal tap showing oligoclonal banding in the spinal fluid
Starting with an initial attack, PPMS is suspected if the disease course progression lasts a year or more. Some doctors prefer to wait a minimum of two years. This leaves the patient in limbo and frustrated, and many of us know that feeling.
What about walking? An MS diagnosis of any kind often brings with it a fear of wheelchairs. Leg involvement of some degree is experienced by MSers of all types and up to 80% of MSers with PPMS, but that is often slow coming on. Seven years after diagnosis, 25% of people with Primary Progressive MS need assistance walking (for instance, a cane). By the 25-year mark, up to 75% of the patients need assistance with walking.
Primary Progressive MS is unique for the following reasons:
- Relapsing/Remitting incidence - The identifying characteristic of Primary Progressive MS is no period of Relapsing/Remitting preceding the progression. When the gradual increase follows a period of Relapsing/Remitting, the diagnosis is Secondary Progressive. When a gradual increase in loss of function is identified as possible MS, the patient is urged to think of anything in the previous twenty years that could possibly be a symptom that just went away. Even one incident can count as a relapse followed by remittance, so the current progression can be labeled Secondary Progressive. If there is no relapse and remittance period, the diagnosis is PPMS.
- Age at diagnosis - MS is usually diagnosed for people between 20 - 50 years old. PPMS is usually identified in the patient’s late 30s or 40s.
Gender ratio - MS affects women more than men. At one time the gender ratio was 2 to 1. In recent years, that ratio has increased to 4 to 1. MS is a woman’s disease. Primary Progressive MS, however, has no such gender bias. Instead, the number of men and women with PPMS is close to equal.
Location of abnormalities and lesions - PPMS begins in the spinal cord rather than the brain like the other MS types. There is a relative lack of brain lesions; that is, fewer lesions showing on the MRI over time, even though the PPMS patient may experience greater disability. There are fewer and smaller cerebral MRI abnormalities. Therefore, a spinal MRI may be invaluable to identify incidental white-matter abnormalities as well as to exclude compression of the spinal cord when eliminating other conditions on the route to a definite MS diagnosis.
- Cognitive difficulties - Because the disease is not centered in the brain, PPMS is less likely to include cognitive difficulties. PPMS is also not inflammatory as in RRMS. Cognitive difficulties in MS are similar to dementia in aging. Often the difference is largely a matter of time needed to process the activity. One study concluded RRMSers are slower than PPMSers and PPMSers are slower than healthy control subjects.
- Disease progression - Because the disease progression of PPMS is more steady than other forms of MS, it curtails the unpredictability factor that disrupts other MSers. An idea about what may be coming allows each person to prepare for it, and to adjust and accept the possibility or reality of disability. This provides a certain feeling of control.
No one can predict with certainty the degree of disability any individual will experience. MS is an individual disease, and one person’s symptom cluster does not necessarily match that of another. The one absolute about Primary Progressive MS is that a relapsing/remitting period did not precede it. All of the other unique characteristics have exceptions. Every person must learn to live with their own version of MS as well as they possibly can.
Because I have no personal experience with Primary Progressive MS, I found stories of people living with it. I hope these help to illustrate the Primary Progressive MS experience.
Eve begins with visits to a chiropractor for back and neck pain, and for dropping a pen when she could no longer hold on. She thought she had a stroke and went through physical therapy. She bought a scroll mouse when she could not use her regular mouse, and the symptoms continued. Eve documented her early symptoms, diagnosis, and ongoing life with Primary Progressive MS.
Shelley Peterman Schwartz
Shelley hosts a regular TV show in Wisconsin where there is a high incidence of MS. She helps us remember that life goes on. Diagnosed as a young mother, Shelley made a great transition from the early dark days following her PPMS diagnosis to encouraging other MSers to enjoy the things they can.
Ken tells the story from a different perspective because the PPMS in his life is that of his wife, Marg. It begins with her diagnosis following a series of strange symptoms. They spent the next decade traveling around the world and have now settled down.
Mimi’s story on WebMD tells about preparing for tomorrow with PPMS.
These stories make it clear there is still life after an MS diagnosis, even if it is for Primary Progressive. Often the progression is very slow. Though there are no remissions, but there are often plateaus of little or no new or worse symptoms, and these may last for months or even years.
Progresssive Relapsing (PRMS)
Between 2-5% of MSers have a steady progression of neurological damage accompanied by periods of relapse, possibly followed by significant remittance. There are two paths to diagnosis for this rare type of MS:
1. It looks like Relapsing/Remitting, but there is accumulated damage between relapses.
2. After a diagnosis of Primary Progressive MS, a relapse occurs and the diagnosis is changed.
Because of this, it is often misdiagnosed, and that is understandable. Essentially, PRMS is Primary Progressive, but with relapses.
A period similar to the Progressive Relapsing type may also ease the change from Relapsing to Secondary Progressive. The Relapsing MSer does not go to bed one night and wake up with Secondary Progressive the next day. Instead there is a transition where symptoms progress but with clear relapses. This description is the same as Progressive Relapsing, but here it was preceded by RRMS.
There is steady progression of functional loss and neurological disorder in both Primary Progressive and Progressive Relapsing MS. These MSers often feel left out in the cold by the medical community. The stories above remind us of how important it is to maintain a high quality of life while living with MS.
Shelley Peterman Schwartz reminds us that for MSers with Primary Progressive “…life will continue to go on in a positive way as long as they have a positive attitude…”
Mimi Mosher says
"accept progression while struggling against it;
find peace and gratitude with what you’ve got"
Notes and Links
Comparison of SPMS & PPMS for assessing responsiveness to therapeutic interventions
MS Network - Learning you can live with PPMS
Description of typical PPMS symptoms
Course of Primary Progressive w/ chart
Course of Progressing Relapsing w/ chart