~Post-Polio
Syndrome~
A
Little History
Polio has been around
for thousands of years. Writings by the Egyptians dating back to 2000
years ago delineate symptoms and signs consistent with acute polio
infection. Dr. Charcoat, a French physician, recognized the symptoms of
both polio and post-polio syndrome in the 1870's. He saw that patients
afflicted with polio later had symptoms of weakness, fatigue, muscle
and joint pain and muscle atrophy after they had reached a stable level
for many years.
In our modern era,
however, even as recent as 1982, polio survivors with the symptoms of
post-polio syndrome were sent to psychiatrists for psychological stress
and emotional disturbances rather than physiological problems. [Gary's note: I can relate to this
misunderstanding in a small way. When I first contracted polio my
doctor thought that I was suffering from some kind of mental block,
rather than a physical problem (see The Uninvited Companion).]
In 1985 a hallmark
meeting was held in Warm Springs, Georgia, the place where President
Roosevelt was treated. That's when we started understanding what
post-polio syndrome is. Since 1985 there has been an explosion of
literature and research in this field.
Fortunately, of those who contracted
polio in the 1940's and 50's, only 1-2% have the central nervous system
symptoms. Many people didn't even know they had had polio, although
they had a high fever, vomiting, headache, neck pain or back pain. That
small 1-2% group, though, had their central nervous system affected,
thus damaging the lower motor neurons within the spinal cord. These are
the nerves that send out their arms and innervate the muscles that
allow us to function in daily activities. A certain percentage also had
bulbar symptoms; that is, the motor neurons located at the lower
portion of the brain, were affected. These individuals developed other
symptoms, such as swallowing difficulty and respiratory dysfunction.
Post Polio
Decades after reaching
a plateau in their recovery from polio 20-40% of the patient population
began experiencing problems now known as post-polio syndrome.
The recovery you
experienced after initial polio onset was due to the resprouting of the
adjacent nerve cells. There was also muscle hypertrophy, i.e., fibers
become stronger and bigger; that's body building. In body building you
do not build new muscle fibers. Rather, a muscle fiber has stress put
on it, for example through weight lifting, and this essentially causes
a breakage in that fiber. As it regenerates it doubles in size,
actually getting two tails instead of having one as before. This is
another reason we recover from acute polio, and we get stronger. Many
who thus conquered polio, may begin experiencing weakness, fatigue, and
pain a few decades later. It seems as if they begin losing the precious
ground they had gained.
Two Things to Note
1) Just because a
patient had an acute paralytic polio infection at a young age and now
has problems with weakness, muscle atrophy or fatigue does not
automatically mean that person has post polio syndrome. PPS is a
diagnosis of exclusion. For example, a patient may have reached a
stable plateau and been doing well functionally for at least 10 years
(most studies say it should be 20 years). Then she begins experiencing
back pain and increased weakness in the lower extremities. These
symptoms could be the result of a nerve being pinched in the back; the
symptoms would present in the same way as post-polio, by reducing
reflexes and causing muscle atrophy, muscle weakness and pain. Numbness
would not necessarily be essential for sensory dysfunction with a nerve
impingement.
2) Not all patients with acute paralytic
polio develop post-polio syndrome.
Risk Factors that Contribute to the
Onset of Post-Polio Syndrome
The median time line
for development of post-polio syndrome is about 35 years. What
determines its development?
- One is the degree of involvement
at the time of onset of acute paralytic polio. If all four limbs were
affected and an iron lung was used for ventilator support, that
generally indicates quite a few of those nerve cells were affected. It
makes sense that if a lot of nerve cells were affected, the surviving
nerve cells have a significantly increased burden put on them
throughout the rest of life. That is a major risk factor.
- Another risk factor is how much
time has elapsed since the acute polio infection. The more time that
has passed the greater the likelihood. [It should be understood that
post-polio studies have been somewhat hindered because much of the data
is retrospective. A lot of the studies have involved sending out
questionnaires, not the best way to collect information. The best study
is prospective: following a group of patients over decades in order to
note changes.]
Post-Polio
Theories
Each motor neuron can
innervate up to 1,000-3,000 muscle fibers, the maximum is 5,000 in the
quadriceps. One neuron in the spinal cord can send its axon all the way
to the peripheral nervous system and innervate the quadriceps. This
neuron can be located at the L-3 and L-4 region of the lumbar area, the
upper lower back. It's sending a long axon all the way over to the
anterior body muscle. This axon has multiple branches which reach out
to innervate approximately 2,000-5,000 muscle fibers.
Two neighboring nerve
cells may each be doing what it needs to do. One gets attacked by the
polio virus, is destroyed and turns to scar tissue. What happens to
it's long arm? It degenerates so that all the branch, what we call the
axon, die. The muscle fibers that it innervated become orphaned; they
lose their mother nerve cell. The muscle fibers start firing
spontaneously without any input from the mother cell, this is the
reason for fasciculations and fibrillations right after their polio
infection. The other cell wants to be neighborly and sends additional
sprouts to the adjacent orphaned muscle fibers.
God was a great
physician, and He still is. Each adjacent nerve cell is programmed to
sprout and innervate up to 8 times it's own limit. A nerve cell
innervating 5,000 muscle fibers can innervate up to 40,000 muscle
fibers. The adjacent muscle cell ends up with a new mom. Unfortunately
it doesn't happen 100%; there is some residual muscle scarring. Unless
a muscle fiber gets new innervation or a new sprout in about a year, it
can become scar tissue. Amazingly, you can lose up to 50% of your motor
nerve cells, essentially neurons, in the spinal cord that are
innervating that muscle and still have near normal muscle strength. So,
you don't need to have all nerves firing and all fibers working to have
normal strength, but you do need some. After polio infection, if enough
nerve cells were affected and killed, muscle fibers were orphaned and
not enough sprouting took place, then muscle atrophy occurs.
So what is post-polio
syndrome? Currently there are two main theories regarding the cause of
post-polio syndrome.
One theory revolves around the effects of
the natural aging process. The
nerve cells that have been attacked with the polio virus and damaged
beyond repair go on to die. Since we are born with the total number of
nerve cells we will ever have, once they die there's no replacing them.
As the years go by, normal aging causes you to lose nerve cells;
exertion, daily activities and the normal degeneration of the spinal
cord causes atrophy and loss of these nerve cells. Normally this causes
no problems since we only need approximately 50-70 % functioning nerve
cells and muscle fibers. This is the reason we usually don't notice
significant weakness until we're about 70. The polio patient, however,
has a reduced reservoir. Normal aging causes more nerve cells to
degenerate and die; weakness becomes more noticeable. If enough nerve
cells were infected by the polio infection, the weakness becomes
significant.
The second theory focuses on the over-use
of the surviving nerve cells. According to the second theory
post-polio syndrome results because the axons that had sprouted and
re-innervated the orphaned muscle fibers begin to die off from
over-fatigue. The muscle fiber, family or clan innervated by one neuron
has shrunk in its size. Axons have died back; the trunk is okay, most
of the branches are okay, but some branches are dying away because of
the increased demand that has been placed on them for the last twenty
plus years. It's not just that the axons are dying away though, the
neuro-muscular junction, which is the point of contact between the arm
of the nerve and and the muscle fiber, is not functioning properly
either. These have been studied under microscopy and we actually note
an alteration at the neuro-muscular junction. In post-polio patients
the neuro-muscular junction is not working properly. Either not enough
acetycholine is being released and/or the receptors are not taking in
the acetylcholine. Acetylcholine released into the junction attaches to
the receptors on the muscle and causes the muscle to contract.
This second theory has
a lot of support. In fact, it is the accepted theory at this point in
time. The hope is that a medication could be developed which would
allow acetycholine to stay in the junction long enough to cause
colonization of this muscle. Studies have been done with Mestinon, but
the research results are mixed.
Effects of
Post-Polio Syndrome
According to responses
to a questionnaire sent out by Mayo Clinic to 1,600 individuals who had
had polio, about 450 respondents could be diagnosed with post-polio
syndrome. The chief complaint of the respondents was fatigue - 79%.
Weakness was the second major complaint - 71%. Other major problems
were joint and muscle pain - 60% and muscle atrophy - 50%. Still others
complained of swallowing difficulty and respiratory dysfunction.
Fatigue
There are two types of
fatigue: generalized fatigue and peripheral fatigue. The symptoms of
general fatigue are similar to flu-like symptoms; you feel tired,
yucky, and don't have much energy. Peripheral fatigue is when you've
used a particular muscle group and afterwards that muscle is weaker
than it was before.
What
causes fatigue?
The body uses more
energy to get the same outcome. You are performing the same activities,
but are weaker and not pacing yourself. There is increased demand on a
weaker muscle. Maybe you've gained some weight, in which case it taking
more energy to accomplish the same tasks. Weakness is caused by fewer
muscle fibers trying to do the same things.
Pain
There also are two
types of pain: muscle pain and joint pain. Muscle pain comes from
lactic acid build-up within the muscle, which causes that muscle to
become less efficient. Pain is the body's way of telling us that we've
done too much. We need to slow down or rest.
The joint pain could be
from many, many things: osteoarthritis, degeneration of the spine,
which can be part of the normal aging process, or it could be
degeneration of the spine or joint because of muscle imbalance. For
example, if my quadriceps are weak, I have to hyper-extend my knee
joint when walking in order to stabilize my knee. Over time the muscles
and the capsule start giving away; hyper-extension can become extremely
painful. Body mechanics are altered when you have muscle imbalance,
which can cause dysfunction. If you have a leg length discrepancy
because of retarded growth on one side, it can actually cause scoliosis.
Muscle atrophy causes
exposure of our nerves. When you cross your legs, your leg goes numb;
the nerve that sits under the lateral aspect of the knee is one of the
more exposed nerves in our body. Micro-trauma over time causes more
numbness. Keep in mind that numbness is not a hallmark of post-polio
syndrome. If you have numbness, there is something else going on and
that something else needs to be looked into.
Muscle
Atrophy
Muscle atrophy, another
hallmark of post-polio syndrome, comes when you have enough of these
muscle fibers losing their axons or their nerve supply. They start
developing scar tissue, which does not have the same volume that a
muscle fiber does.
Suggestions for Dealing with Post-Polio
Syndrome
-
Be as active as possible. Polio survivors should be as active as
possible, but you have to be logical about it! You definitely have to
know what your body's limitations are. You don't want to stop all
exercise, that's counter productive, because of disuse atrophy. You
need to be as active as possible, but the activity you do should be
paced.
- Sub-max exercises 3 times a week
are very helpful. The key word is sub-max. Don't exercise until you're
tired. Stop before you get tired. Low impact aerobic conditioning is
extremely important. Stretching, non-fatiguing exercises and keeping in
good cardio-pulmonary condition are most important.
Muscle cramps or a
charley horse can be extremely painful. If you overdo an activity, you
have spasms and fasiculations, maybe at night when you're trying to
sleep. That generally indicates muscles have been pushed beyond what
they were able to tolerate. Back off somewhat from what you were doing
before. Generally speaking, post-polio individuals have a reputation
for being highly educated, very stubborn, extremely independent, and in
some respects, just plain difficult. Most of us are Type A
personalities. Slowing down, pacing themselves is just not natural to
them (me!).
- LISTEN TO YOUR BODY, Be as
educated about the disease process as possible, and be sure you
actually have post-polio Syndrome and not something else that would be
tested differently.
- As post-polio syndrome develops one must
a balanced life. Fatigue is a major symptom, but it is also a symptom
seen in depression. Counseling may be needed to help determine what
part of the fatigue is physical and what part may have an emotional
component.
- Part of the components in
relaxation and stress management are not only prioritizing the things
that you do, pacing yourself, exercise and rest. It is vital that you
look at life holistically: mentally, physically, and spiritually.
Physically you need to learn to relax the body so muscles can work more
efficiently for you. We have to mentally release thoughts, worries and
concerns and mentally become very, very calm. Spiritually we need to
have a relationship with God that allows us to draw from Him all the
resources He makes available to us: prayer, guidance, peace, renewal,
joy, hope, life to the fullest. Pay attention to your life physically,
mentally and spiritually.
----------------------
A
brief comment bout fibromyalgia syndrome, which is diffused pain. It
consists of diffused muscle aches. It appears to be present in about
10-15% of patients suffering from post-polio syndrome. It does affect
women in about 80% of the cases. It presents as a myriad of complex
symptoms and signs, but it's also a diagnosis of exclusion. Just
because you have post-polio syndrome doesn't mean you can't have
something else right along with it. It can present with fatigue,
difficulty sleeping, generalized muscle aches and joint pains and
reduced ability to function.
This
article based on and adapted from a lecture given April 12, 1997 by
Hooman Sedighi, Physiatrist, Director of Post-Polio Clinic at
HealthSouth, Dallas, Texas. I have tried to be true to the original
message, but have condensed and simplified it.
|