Now called INTERNATIONAL POLIO NETWORK Post-PoZi~Health WINTER 1995 + VOL. 11, NO. 1 Sixth International Post-Polio and Independent Living Conference JUNE 1994 Defining Post-Polio Problems Jacquelin Perry, MD, Pathookinesiology, some damage. It has been demonstrated that as muscles Rancho Los Amigos Medical Center, Downey, California contract they need time to repair and refuel. If the mus- cles are functioning almost continually, they do not have The term late effects of polio is the umbrella over three time to repair. Muscles ahd their ability to function are subdivisions. the main problem. The cause of post-polio problems is neuromusculoskele- Another problem is the neuromuscular junction. This is tal failure from chronic overuse of a polio altered sys- evidenced by information from Gunnar Grimby, MD, tem. How has that system been altered? The pathology PhD, Sahlgren Hospital, GGteborg, Sweden. He studied of polio involves destruction of the anterior horn cell, the anterior tibialis and demonstrated that a 3+ muscle, and the only functional residual is weakness. New polio which is about 25% of normal on the Beasely scale, has problems are the effects of the functioning of muscle marked hypertrophy (enlargement) compared to a 4+ which had less than normal capability muscle which would be about a 45% muscle. Both of So, who's under the umbrella? First are the asympto- them have degrees of weakness, but the 3+ is much matic post-polio individuals who have a known history more weak than the 4+. of polio but who are not having troubles now. Their Grimby also found that the 3+ tibialis anterior was not lifestyle and their physical ability are balanced. These only markedly hypertrophied, but its activational rate persons can, for example, go on running and continue was twice normal. One gains new or additional strength what they are doing as long as they have no symptoms. either by activating more muscle fibers or making the If symptoms do occur, it is time to become more cau- current muscle fibers work twice as hard. In 3+ muscles tious about their activities. there are not very many muscle fibers, so they are The second group has symptoms which indicate the worked twice as hard to meet the functional demand. lifestyle demands now exceed their muscles' ability to The individuals most difficult to get in balance are the meet those demands. The symptoms of pain, fatigue, ones with 3+ and 2 muscles all over. Their muscles are and new weakness are signs of overuse with a penalty not strong enough to function without overtime use. We have a list of about 1,000 polio survivors and of that The third group, which we often overlook, is the polio group we found 107 who thought they had full recovery. survivor with joint degeneration problems. It is just Eighty nine percent of them lived very active lives while plain wear and tear, either from substitutive posture 24% of them had been in athletics. They came to the used to replace inadequate muscles, or by the impact of clinic because they were having symptoms. Ninety three loading a joint instead of letting it yield on the muscles. percent showed weakness with at least one muscle less For instance, a hyper-extended knee causes bones to than grade 5. (See chart below.) hit together with every step, and the joint becomes When a muscle creates a force to control or create degenerative and arthritic. Joint degeneration problems motion, it also creates a force internally and can cause do not result in muscle pain. There is pain and tender- ness in the specific joint, resulting in deformity and x-ray changes. Beasely Scale Again, feeling that the primary cause is overuse, the Muscle Grade % of True Normal principal management is lifestyle modification. No mat- ter what else we do, we find that about 95% of people 5 Normal 50% have to make lifestyle changes in order to make exercise 4 Good 40% or other devices work. 3 Fair 20°h Out of 250 patients we have studied quantitatively on 2 Poor 10% a scale of "better, same, worse." The majority of them 1 Trace 1% were "better," meaning that they lost their symptoms of I I continued on page 2 Lour0 S. Halstead, MD, Physical Medicine and Defining Post-Polio Problems continued from page 1 Rehabilitation, National Rehabilitation Hospital, Washington, D. C. pain and fatigue by modifying their lifestyle. A small percentage did not make a change because they reject From the point of view of a clinician who sees polio the whole idea of lifestyle modification. Some became individuals in a clinic, "What diagnosis do I use?" often worse and are the individuals with all muscles of grades makes a great deal of difference, because of the implica- 2 and 3. We have trouble finding a lifestyle for them tions for insurance and disability payments. We do not which does not cause any strain. have enough information, insight, or wisdom to sepa- rate specific definitions except for clinical use. Neil Cashman, M D, Neurology, The three major diagnostic terms - the late effects of Montreal Neurological Institute, Montreal, Quebec polio, post-polio syndrome, and post-polio muscular atrophy - can be described by imagining three concen- Today, there is no consensus, no position paper, no diag- tric circles. A large outer circle labeled the late effects nostic test, no agreed-upon criteria about this syndrome, of polio represents a grab bag. Anyone who has serious so there will be differences of opinion and some overlap. involvement with a neuromuscular disease, and it does not have to be polio, will experience a variety of prob- From a neurologist's point of view, post-polio syndrome lems, if they live long enough. has to begin in the spinal cord and brain stem with the original infection and destruction of motor neurons The late effects of polio refers to a group of symptoms causing paralytic polio. A common question arises: "I and signs which people who had polio many years had non-paralytic polio. Am I susceptible to the post- ago now experience and include weakness, fatigue, polio syndrome?" A neurologist would have to say "no." muscle pain, joint pain, decreased endurance, and new There is no evidence that people who had no infection atrophy They also include increased weight gain, osteo- of motor neurons can develop classical post-post syn- porosis, increased risk of fractures, scoliosis, increase drome. In fact, the worse the paralytic poliomyelitis, the in pulmonary problems, sleep difficulties, and psycho- more likely new symptoms are to develop. logical problems. Another common question is: "Am I out of the woods People can experience a whole list of problems that are with post-polio syndrome after 40-years?" Burk Jubelt, in this big circle as a result of having had polio, but they MD, (SUNY, Syracuse, NY), and I reviewed every do not have post-polio syndrome. recorded case of post-polio syndrome that had been Inside the big circle is a smaller circle labeled post-polio reported up to 1987 and found the mean onset was 36 syndrome. More narrowly defined, a diagnosis of post- years. Some people develop new symptoms 10 years polio syndrome requires the presence of new neurogenic after polio, and others develop symptoms 60 years after weakness. This new weakness is caused by some dys- polio. Unfortunately, there is no out-of-the-woods function of the motor neuron that was originally affect- threshold age. ed. Post-polio syndrome is a neurologic disease which There is a running controversy in the medical literature occurs as a result of having had an invasion of the polio of "lumpers" and "splitters." Some authorities like to virus to part of the nervous system many years earlier. put diseases together under certain rubrics, and others like to break off certain diseases. My opinion is that it is There are five elements to make the too early to split sub-syndromes off the post-polio syn- diagnosis of post-polio syndrome: drome. My conservatism, in part, is based on work with I. a history of paralytic polio; people who had post-polio muscular atrophy People 2. a fair to good recovery (something had to recover with atrophy were just as likely to have pain and fatigue to have loss later on); as people without new atrophy There is not enough 3. a period of neurologic stability; data to make a distinction and say this is merely muscu- loskeletal; that is neurogenic. They act differently and, 4. onset of new weakness; and they are treated differently 5. exclusion of other causes for the new weakness. The original meaning of the word "syndrome" comes from the Greek - "running together." These symptoms Currently, making a diagnosis is difficult because there present in polio persons time after time, and the symp- is no diagnostic test; unfortunately at this stage, diagno- toms really do run together. There is something going sis is still primarily by history and physical examination. on and, in my opinion, it is too early to dogmatize The smallest and most inner circle is post-polio muscu- sub-groups. lar atrophy and is reasonably straightforward. It is post- The safest term, to date, is post-polio syndrome, and polio syndrome in someone with new muscle atrophy the safest definition of the post-polio syndrome is a Another better term that might be used for these last new clinical syndrome of pain, weakness, and fatigue two diagnoses is post-polio motor neuron disease in individuals who recovered from acute paralytic which focuses on the motor neuron as the primary poliomyelitis.