Diagnosing Secondary Progressive MS
Prior to the availability of the approved disease-modifying therapies (DMTs), studies
indicated that 50% of those diagnosed with relapsing-remitting MS (RRMS) would
transition to secondary-progressive MS (SPMS) within 10 years, and 90% would
transition within 25 years.|
History does indicate that the majority of people with multiple sclerosis (MS) will
eventually transition from a relapsing-remitting disease course to a more progressive
one. While the DMTs help to reduce the number and severity of relapses (attacks or
exacerbations) in relapsing forms of MS. These therapies seem to have an impact on
disease progression, buy they are still unable to halt the progression completely.
It's still too soon, however, to see the specific extent to which the DMTs have
changed or more importantly delayed the transition to SPMS.
SPMS can begin but only after an initial relapsing-remitting course. History says that
most people that are diagnosed with RRMS will eventually transition to a secondary
progressive course. SPMS shows that there is a progressive worsening of neurologic
function over time. SPMS can be further characterized at different points in time as
either active or not active, as well as with progression or without progression.
SPMS occurs in individuals who initially had a relapsing-remitting disease course. In
other words, SPMS occurs as a second phase of the disease for many individuals.
Primary-progressive MS (PPMS) stands on its own as the first and only phase of the
illness for approximately 15% of those with MS.
Now if you've just been diagnosed with SPMS but never RRMS, chances are that you most
likely you have had a relapsing-remitting form for some time and just didn't know it.
If all of the symptoms that you have had prior to this time were mild enough to not be
noticed, then you have had RRMS but not in any manner as to make you think that something
has been wrong. It finally becomes noticable when you may begin to feel a shift in your
disease and its symptoms begin to present themself with a big "hello".
Now having essentially skipped RRMS can be a double-edged sword in a manner of
speaking in that you were lucky enough not to be aware of having MS for some time
and not dealing with any treatments. On the other side is the fact that if you had
known earlier and started treatment then, it may not have progressed into SPMS quite
as fast or aggressively. But as with everything else in life, who really knows what
would be different?
With SPMS, those diagnosed may or may not continue to experience relapses caused by
inflammation. This inflammation that occurs early in the MS disease process, and is
the trademark of RRMS, slowly lessens over time. With fewer inflammatory changes
occuring, it will present itself with fewer to no relapses. This lack of relapes might
make a person feel that everything is getting better, except a gradual worsening of
symptoms may become common over time as it transitions into a steadily progressive
phase. All of this worsening is known as disease progression and will typically be
characterized by nerve damage or loss.
Because the transition from a relapsing-remitting course to a more progressive one is a
gradual process, your physician will not be able to tell exactly when it's happening. If
a person’s symptoms are worsening, the challenge for the doctor is to determine whether:
||The worsening is left over from the last relapse (permanent but
stable damage that remains after the inflammatory attack has ended) indicating
that the person is experiencing an RRMS disease course; or
||The disease is continuing to worsen even though the person is
no longer experiencing inflammatory relapses indicating that the person
has transitioned to a SPMS disease course.
You might be wondering how an exact determination or diagnosis can be made as to whether
or not you have SPMS. Unfortunately, this diagnosis is very similar to an initial diagnosis.
The diagnosis consists of several methods, including a careful history of the changes
in a person’s symptoms (which was probably not available for the RRMS diagnosis), a
neurologic examination, and repeat magnetic resonance imaging (MRI) scans, will all help
to determine whether the transition to SPMS has occurred or not.
Disease activity and progression should be evaluated at least yearly by neurologic
examination and MRI. Being able to characterize the course of your disease at different
points in time will help you and your doctor discuss your treatment options and expected
outcome. If you have SPMS that is active, you can still maintain a treatment with a
disease-modifying therapy approved for SPMS to reduce the risk of a relapse.
||If you have SPMS that is active and progressing in spite of the
medication you are taking, the conversation with your MS care provider might
be about the potential benefits and risks associated with switching to a more
aggressive treatment strategy.
||If your SPMS is not active but there is evidence of progression
and accumulation of disability, you and your MS care provider will want to
focus on rehabilitation strategies to help improve your function and mobility,
and promote safety and independence.
||If your SPMS is stable without activity or progression, the
conversation with your MS care could focus on rehabilitation and other symptom
management strategies to help you maintain function.
Similar symptoms exist within all forms of MS. But SPMS symptoms are usually worse
than RRMS. During the early stages of RRMS, symptoms are noticeable, but they aren’t
necessarily severe enough to interfere with your everyday activities. Once MS progresses
to the secondary-progressive stage, symptoms become more noticable.
RRMS can be unpredictable, but there’s usually a pattern of clear attacks followed by
times of recovery. With SPMS, relapses tend to be less noticable or distinct. They may
also happen less often or not at all. However, when you do have relapses, recovery is
typically not as complete.
Symptoms of SPMS are similar to those of PPMS. The main symptom of both forms of the
condition is gradual worsening of disability.
This may be experienced through increased:
||Numbness or tingling
||Vision problems, such as double vision
||Spasticity or stiffness of the muscles
||Bowel and bladder problems, such as urgent need to urinate
||Problems with cognition, such as learning and memory or information processing
||Difficulty with walking and coordination
Treatment for Progressive Forms of MS
Treatment of SPMS is more difficult than relapsing MS. Although DMTs were found
in some trials to prevent progression of disability in SPMS, the effect was
modest and seen primarily in those subjects with superimposed active inflammation.
Those studies have shown that it's worthwhile to use anti-inflammatory medications
during this stage if there is evidence of persistent active inflammation, either
clinically or radiologically, and if the side effects can be tolerated. Those
studies also lean on the side of caution in making sure that reasonable
expectations are kept in check.
The interferon beta medications (Avonex, Betaseron, Extavia, Plegridy and Rebif),
Aubagio (teriflunomide), Gilenya (fingolimod), Lemtrada (alemtuzumab),Tecfidera
(dimethyl fumarate), and Tysabri (natalizumab) are all approved by the FDA for
use in relapsing forms of MS, which include those individuals who have
transitioned to SPMS but continue to have relapses and/or evidence of disease
activity on MRI. Therefore, a person who has been on one of these medications
during the initial relapsing-remitting phase of the disease will likely continue
on it unless his or her physician feels that it's no longer doing an adequate
job of controlling disease activity. At that time, the physician will likely
recommend changing to another one of these medications or to Mitoxantrone,
a chemotherapeutic agent that has been approved by the FDA specifically for
SPMS, as well as worsening relapsing MS.