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~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.

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    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.


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