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Management of postpolio syndrome

Author(s): Gonzalez H, Olsson T, Borg K
Affiliation(s): Division of Rehabilitation Medicine, Department of Clinical Sciences, Danderyd Hospital, Karolinska Institute, Stockholm, Sweden – [email protected]

Publication(s): The Lancet Neurology
Citation: Lancet Neurol. 2010 Jun; 9(6):634-42 and Comment in: Lancet Neurol. 2010 Jun; 9(6):561-3

Postpolio syndrome is characterised by the exacerbation of existing or new health problems, most often muscle weakness and fatigability, general fatigue, and pain, after a period of stability subsequent to acute polio infection. Diagnosis is based on the presence of a lower motor neuron disorder that is supported by neurophysiological findings, with exclusion of other disorders as causes of the new symptoms. The muscle-related effects of postpolio syndrome are possibly associated with an ongoing process of denervation and reinnervation, reaching a point at which denervation is no longer compensated for by reinnervation. The cause of this denervation is unknown, but an inflammatory process is possible. Rehabilitation in patients with postpolio syndrome should take a multiprofessional and multidisciplinary approach, with an emphasis on physiotherapy, including enhanced or individually modified physical activity, and muscle training. Patients with postpolio syndrome should be advised to avoid both inactivity and overuse of weak muscles. Evaluation of the need for orthoses and assistive devices is often required.

Postpolio syndrome: unanswered questions regarding cause, course, risk factors, and therapies

Author(s): Nollet F
Affiliation(s): Not stated

Publication(s): The Lancet Neurology
Citation: Lancet Neurol. 2010 Jun;9(6):561-3 - Comment on: Lancet Neurol. 2010 Jun;9(6):634-42

Living with the consequences of poliomyelitis is not recognised as an important health issue at present. However, millions of people worldwide have lasting impairments caused by polio infection, many of whom also had a decline in muscle function and decline in activities of daily living after years of stable functioning.

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Aging and sequelae of poliomyelitis

Author(s): Laffont I, Julia M, Tiffreau V, Yelnik A, Herisson C, Pelissier J
Affiliation(s): centre hospitalier régional universitaire de Montpellier, hôpital Lapeyronie, France – [email protected]

Publication(s): Annals of Physical and Rehabilitation Medicine
Citation: Ann Phys Rehabil Med. 2010 Feb;53(1):24-33 – Epub 2009 Nov 10

OBJECTIVE: We estimate that there are about 50,000 persons who survived poliomyelitis in their childhood in France (mean age estimated between 50 and 65 years). After a few decades of stability, 30 to 65% of individuals who had been infected and recovered from polio begin to experience new signs and symptoms.

METHOD: Review of the literature on Pubmed with the following keywords "Poliomyelitis" and "Post-Polio Syndrome (PPS)".

RESULTS: These new signs and symptoms are characterized by muscular atrophy (decreased muscle mass), muscle weakness and fatigue, muscle and/or joint pain. All these symptoms lead to significant changes in mobility with falls and inability to carry on with daily life activities. There are several intricate causes. The normal aging process and weight gain are regularly blamed. Respiratory disorders and sleep disorders must be looked for: respiratory insufficiency, sleep-related breathing disorders such as sleep apnea, restless legs syndrome. Orthopedics complications are quite common: soft-tissue pathologies of the upper limbs, degenerative pathologies of the large joints or spinal cord, fall-related fractures. Finally, the onset of an authentic PPS is possible.

CONCLUSION: The therapeutic care of this late functional deterioration requires regular monitoring check-ups in order to implement preventive measures and appropriate treatment. This therapeutic care must be multidisciplinary as physical rehabilitation, orthotics and technical aids are all essential.

Actual and perceived activity levels in polio survivors and older controls: a longitudinal study

Author(s): Klein MG, Braitman LE, Costello R, Keenan MA, Esquenazi A
Affiliation(s): Moss Rehabilitation Research Institute, Philadelphia, PA; Thomas Jefferson University, Philadelphia 19141, USA – [email protected]

Publication(s): Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 2008 Feb; 89(2):297-303

OBJECTIVE: To examine factors associated with daily step activity, perceived activity, maximum walking speed, and walking speed reserve over time in polio survivors and older adults with no history of polio.

DESIGN: Longitudinal study.

SETTING: A research clinic and the community.

PARTICIPANTS: Polio survivors (n=96; 65 in postpolio syndrome [PPS] group, 31 in non-PPS group) and older adults (n=112) with no history of polio.

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Daily step activity, perceived activity, maximum walking speed, and walking speed reserve.

RESULTS: Results showed decreases in perceived activity over time in the PPS group. However, there was no change in average daily walking activity. Overall, polio survivors walk less and have a smaller walking speed reserve than controls. Knee strength was positively associated with maximum walking speed and walking speed reserve in all groups. Weight and age were associated with daily step activity in controls but not polio survivors.

CONCLUSIONS: Daily walking activity did not change statistically over the 3-year study period, although perceived activity and the walking speed reserve decreased among polio survivors with PPS. On average, polio survivors appear to function with minimal functional reserve, as their preferred walking speed was close to their maximum speed.

Franklin D Roosevelt’s Shangri-La: Foreshadowing the Independent Living Movement in Warm Springs, Georgia, 1926-1945

Author(s): Holland, D
Affiliation(s): Department of Psychology, University of Arkansas, Little Rock, AR 72204, USA

Publication(s): Disability & Society
Citation: Disability & Society. 2006 Aug; 21(5):513-35

American President Franklin Delano Roosevelt is well known to have disguised and minimized his disability in his role as a political leader. Less well known is the remarkable nature of the colony he established for people with disabilities from polio in Warm Springs, Georgia in the 1920s, 1930s and 1940s. The colony at Warm Springs represents a unique historical community in which disability was not stigmatized; where people with disabilities controlled their own resources and their own lives; and where the medical model of disability was repudiated. As such, the Warm Springs community represents a remarkable period and place in disability history that warrants continued study. New evidence drawn from the archives of the Roosevelt Warm Springs Institute for Rehabilitation, the FDR Presidential Library in Hyde Park, New York and the personal scrapbooks of former residents of the Warm Springs colony provides further support for the theory that FDR's Warm Springs colony represented an early precursor to the philosophies and values promoted by the Independent Living Movement that emerged 50 years later. The Warm Springs colony offered an unprecedented approach to rehabilitation and independent living for people with disabilities from polio in the 1920s, 1930s and 1940s, and because of this provides an invaluable lesson from history that deserves ongoing attention.

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EFNS guideline on diagnosis and management of post-polio syndrome - Report of an EFNS task force

Author(s): Farbu E, Gilhus NE, Barnes MP, Borg K, de Visser M, Driessen A, Howard R, Nollet F, Opara J, Stalberg E
Affiliation(s): Department of Neurology, Haukeland University Hospital, University of Bergen, Bergen, Norway – [email protected] – European Federation of Neurological Society

Publication(s): European Journal of Neurology
Citation: Eur J Neurol. 2006 Aug; 13(8):795-801

Post-polio syndrome (PPS) is characterized by new or increased muscular weakness, atrophy, muscle pain and fatigue several years after acute polio. The aim of the article is to prepare diagnostic criteria for PPS, and to evaluate the existing evidence for therapeutic interventions. The Medline, EMBASE and ISI databases were searched. Consensus in the group was reached after discussion by e-mail. We recommend Halstead's definition of PPS from 1991 as diagnostic criteria. Supervised, aerobic muscular training, both isokinetic and isometric, is a safe and effective way to prevent further decline for patients with moderate weakness (Level B). Muscular training can also improve muscular fatigue, muscle weakness and pain. Training in a warm climate and non-swimming water exercises are particularly useful (Level B). Respiratory muscle training can improve pulmonary function. Recognition of respiratory impairment and early introduction of non-invasive ventilatory aids prevent or delay further respiratory decline and the need for invasive respiratory aid (Level C). Group training, regular follow-up and patient education are useful for the patients' mental status and well-being. Weight loss, adjustment and introduction of properly fitted assistive devices should be considered (good practice points). A small number of controlled studies of potential-specific treatments for PPS have been completed, but no definitive therapeutic effect has been reported for the agents evaluated (pyridostigmine, corticosteroids, amantadine). Future randomized trials should particularly address the treatment of pain, which is commonly reported by PPS patients. There is also a need for studies evaluating the long-term effects of muscular training.

Postpolio syndrome and anesthesia

Author(s): Lambert DA, Giannouli E, Schmidt BJ
Affiliation(s): Department of Anesthesia, University of Manitoba, Canada

Publication(s): Journal of the American Society of Anesthesiologists
Citation: Anesthesiology. 2005 Sep;103(3):638-44

The development of polio vaccines 50 years ago essentially halted childhood polio epidemics in the industrialized world. During the past quarter century, a constellation of delayed neuromuscular symptoms, called postpolio syndrome, became recognized among the aging polio survivors. The prevalence of postpolio syndrome in the US population is estimated to be in the hundreds of thousands. The most common symptoms are fatigue, pain, and new onset weakness thought to be related to delayed deterioration of motor neuron function. When a patient with postpolio syndrome presents for surgery, special precautions are warranted, because these patients may have respiratory impairment, sleep apnea, swallowing difficulties, and cold intolerance. This article first reviews clinical features and some pathoetiologic theories of postpolio syndrome and then focuses on anesthetic considerations including the use of common anesthetics, neuromuscular blockade, regional anesthesia, and general anesthetic management strategies.

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The course of functional status and muscle strength in patients with late-onset sequelae of poliomyelitis: a systematic review

Author(s): Stolwijk-Swüste JM, Beelen A, Lankhorst GJ, Nollet F; CARPA Study Group
Affiliation(s): Department of Rehabilitation Medicine, VU University Medical Center, Amsterdam, The Netherlands – [email protected]

Publication(s): Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 2005 Aug;86(8):1693-701

OBJECTIVES: To review systematically studies of late-onset polio sequelae on the course of functional status and muscle strength over time and to identify prognostic factors of change.

DATA SOURCES: We conducted a computerized literature search up to July 2004 in MEDLINE, EMBASE, CINAHL, Web of Science, PsychInfo, and the Cochrane controlled trial register using the key words: postpolio, postpoliomyelitis, postpoliomyelitis syndrome, post poliomyelitis muscular atrophy, and poliomyelitis.

STUDY SELECTION: Reports were selected by 1 reviewer if the study involved subjects with a history of poliomyelitis, the outcome measures described functional status or muscle strength, and follow-up was for at least 6 months.

DATA EXTRACTION: Studies were summarized with regard to population, design, sample size, outcome measures, results, and methodologic scores. Overlap in populations between studies was checked.

DATA SYNTHESIS: Of 71 potentially relevant studies, 19 were included (2 on functional status, 15 on muscle strength, 2 on both muscle strength and functional status). Two studies on the course of functional status had sufficient quality and reported inconsistent results. Four studies on the course of muscle strength had sufficient quality. Two studies reported a decline in strength and 2 reported no change. Decline in strength was only reported in studies with a follow-up period longer than 2 years. One study reported extent of paresis as a prognostic factor for change in perceived physical mobility.

CONCLUSIONS: Conclusions cannot be drawn from the literature with regard to the functional course or prognostic factors in late-onset polio sequelae. The rate of decline in muscle strength is slow, and prognostic factors have not yet been identified. Long-term follow-up studies with unselected study populations and age-matched controls are needed, with specific focus on prognostic factors.

Post-poliomyelitis syndrome

Author(s): Trojan DA, Cashman NR
Affiliation(s): Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, 3801 rue Université, Montréal, Québec H3A 2B4, Canada – [email protected]

Publication(s): Muscle & Nerve
Citation: Muscle Nerve. 2005 Jan;31(1):6-19

Post-poliomyelitis syndrome (PPS) is a common neurological disorder that occurs in a large proportion of individuals who have recovered from paralytic poliomyelitis. The main clinical features are new weakness, muscular fatigability, general fatigue, and pain. The primary criteria necessary for the diagnosis of PPS are a history of paralytic poliomyelitis, partial or complete recovery of neurological function followed by a period of stability (usually several decades), persistent new muscle weakness or abnormal muscle fatigability, and the exclusion of other causes of new symptoms. The cause of PPS remains unclear, but is likely due to a distal degeneration of enlarged post-poliomyelitis motor units. Contributing factors to PPS may be aging (with motor neuron loss), overuse, and disuse. PPS is usually a slowly progressive neuromuscular disease. Although there is no specific treatment for PPS, an interdisciplinary management program can be useful in controlling symptoms.

Postpolio syndrome

Author(s): Nollet F, de Visser M
Affiliation(s): Department of Rehabilitation, Academic Medical Center, University of Amsterdam, PO Box 22660, 1100 DD Amsterdam, the Netherlands – [email protected]

Publication(s): Archives of Neurology
Citation: Arch Neurol. 2004 Jul;61(7):1142-4

This paper has no abstract - this is an extract:

Postpolio syndrome (PPS) refers to a decline of muscle function usually occurring 30 to 40 years after the acute polio episode. This syndrome has been widely recognized only during the last decades, when many people affected by the large epidemics of the previous century experienced new muscle weakness as they grew older. However, cases of late-onset weakening following poliomyelitis were already reported at the end of the 19th century.

Prior poliomyelitis – IVIg treatment reduces proinflammatory cytokine production

Author(s): Gonzalez H, Khademi M, Andersson M, Piehl F, Wallström E, Borg K, Olsson T
Affiliation(s): Department of Neuroimmunology Unit, Center for Molecular Medicine, Karolinska Hospital, Stockholm, Sweden – [email protected]

Publication(s): Journal of Neuroimmunology
Citation: J Neuroimmunol. 2004 May; 150(1-2):139-44

The postpolio syndrome (PPS) is characterized by progressive disabilities decades after recovery from the acute paralytic disease. There are reports on intrathecal inflammatory reactions in PPS, including increased expression of cytokines by cerebrospinal fluid (CSF) mononuclear cells (CSF-MC). This is potentially of relevance for the clinical condition. We here explored if cytokine expression in the CSF of PPS patients could be modulated by high-dose intravenous immunoglobulins (IvIg). The expression of TNF-alpha, IFN-gamma, IL-10 and IL-4 mRNAs was measured by real-time RT-PCR in CSF and peripheral blood mononuclear cells (PBMC) of 16 PPS patients before, and 6-8 weeks after IvIg treatment, and in 26 patients with noninflammatory other neurological diseases (OND). TNF-alpha, IFN-gamma and IL-10 CSF mRNA levels were elevated in samples from untreated persons with PPS compared to OND. Upon IvIg treatment, IFN-gamma and TNF-alpha mRNA levels were dramatically reduced, while IL-10 remained unchanged. Placebo-controlled studies are now warranted to evaluate if IvIg treatment also has any effects on the clinical manifestations of PPS.

Prior poliomyelitis - evidence of cytokine production in the central nervous system

Author(s): Gonzalez H, Khademi M, Andersson M, Wallström E, Borg K, Olsson T
Affiliation(s): Department of Clinical Neuroscience, Division of Neurology, Karolinska Hospital, S-171 76 Stockholm, Sweden – [email protected]

Publication(s): Journal of the Neurological Sciences
Citation: J Neurol Sci. 2002 Dec 15; 205(1):9-13 and Comment in: J Neurol Sci. 2002 Dec 15; 205(1):5-8

In order to study the role of a possible inflammatory reaction in the post-polio syndrome (PPS) four key cytokines were determined by means of mRNA expression in mononuclear cells from cerebrospinal fluid (CSF) and peripheral blood of 13 patients. Data were compared with those of samples from eight non-inflammatory control persons. The PPS-patients displayed increased numbers of CSF cells expressing mRNA for TNF-alpha (p<0.02), IFN-gamma (p<0.02), IL-4 (p<0.001) and IL-10 (p<0.05), in comparison to the non-inflammatory controls. As positive controls, samples from patients with Multiple Sclerosis (MS) were examined. We conclude that there is a chronic intra CNS expression of inflammatory cytokines in PPS, in the range of that in MS, a well known neuroinflammatory disease. However, the pathogenic significance of this is unclear.

Characteristics and management of postpolio syndrome

Author(s): Jubelt B, Agre JC
Affiliation(s): State University of New York, Upstate Medical University, 750 E Adams St, Syracuse, NY 13210, USA – [email protected]

Publication(s): The Journal of the American Medical Association
Citation: JAMA. 2000 Jul 26;284(4):412-4

Postpolio syndrome (PPS) refers to new, late manifestations occurring many years after acute poliomyelitis infection. Over the last 25 years, PPS has become a relatively common problem encountered by primary care physicians. A 1987 National Health Interview Survey estimated that about half of the 640,000 survivors of paralytic poliomyelitis in the United States had new late manifestations of PPS. Subsequent studies in the 1990s have found the occurrence of PPS among patients with previous poliomyelitis to range from 28.5% to 64%. The average time in various reports from the acute poliomyelitis until the onset of PPS is about 35 years, with a range from 8 to 71 years. However, it is unclear if the occurrence of PPS increases with aging, which may be the case based on the most accepted etiologic hypothesis.

An 8-year longitudinal study of muscle strength, muscle fiber size, and dynamic electromyogram in individuals with late polio

Author(s): Grimby G, Stålberg E, Sandberg A, Sunnerhagen KS
Affiliation(s): Department of Rehabilitation Medicine, Sahlgrenska University Hospital, Göteborg University, Sweden

Publication(s): Muscle & Nerve
Citation: Muscle Nerve. 1998 Nov; 21(11):1428-37

Twenty-one subjects with polio 24 to 51 years prior to the first examination were studied on three occasions, each 4 years apart with measurements of muscle strength and endurance for knee extension, macro EMG, and muscle biopsy from vastus lateralis. On average the muscle strength decreased during the 8-year follow-up by 9-15%. Endurance decreased during the observation period. The muscle fiber area was markedly increased in most subjects. There was a decrease in the capillarization during the follow-up. Macro EMG was increased in all subjects (range 3-42 times control) and increased in 20 legs during the 8-year follow-up, but showed a decrease in 8 of 9 legs with an approximative breakpoint when macro MUPs were around 20 times the normal size. Thus, evidence of on-going denervation/reinnervation as well as of failing capacity to maintain large motor units was demonstrated. SFEMG showed a moderate degree of disturbed neuromuscular transmission.

Management of post-polio syndrome

Author(s): Trojan DA, Finch L
Affiliation(s): Not stated

Publication(s): NeuroRehabilitation: An Interdisciplinary Journal
Citation: NeuroRehabil. 1997;8:93-105

The management of patients presenting with post- poliomyelitis syndrome is discussed. It is essential to identify and treat other medical and neurological conditions which could produce these symptoms. New weakness can be managed with exercise, avoidance of muscular overuse, weight loss, orthoses and assistive devices. Fatigue can be managed with energy conservation techniques. The management of pain is dependent upon its causes. Treatments are reviewed.

Strength, endurance, and work capacity after muscle strengthening exercise in postpolio subjects

Author(s): Agre JC, Rodriquez AA, Franke TM
Affiliation(s): Department of Rehabilitation Medicine, University of Wisconsin Medical School, Madison, USA

Publication(s): Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 1997 Jul; 78(7):681-6

OBJECTIVE: To determine whether a 12-week home quadriceps muscle strengthening exercise program would increase muscle strength, isometric endurance, and tension time index (TTI) in postpolio syndrome subjects without adversely affecting the surviving motor units or the muscle.

DESIGN: A longitudinal study to investigate the effect of a 12-week exercise program on neuromuscular function and electromyographic variables.

SETTING: Neuromuscular laboratory of a university hospital.

SUBJECTS: Seven subjects were recruited from a cohort of 12 subjects who had participated in a previous exercise study. All subjects had greater than antigravity strength of the quadriceps. Upon completion of a postpolio questionnaire, all acknowledged common postpolio syndrome symptoms such as new fatigue, pain, and weakness; 6 of the 7 acknowledged new strength decline.

INTERVENTION: On Mondays and Thursdays subjects performed three sets of four maximal isometric contractions of the quadriceps held for 5 seconds each. On Tuesdays and Fridays subjects performed three sets of 12 dynamic knee extension exercises with ankle weights.

MAIN OUTCOME MEASURES: Neuromuscular variables of the quadriceps muscles were measured at the beginning and completion of the exercise program and included: isokinetic peak torque (ISOKPT, at 60 degrees/sec angular velocity) and total work performed of four contractions (ISOKTW), isometric peak torque (MVC), endurance (EDUR, time subject could hold isometric contraction at 40% of the initial MVC), isometric tension time index (TTI, product of endurance time and torque at 40% of MVC), and initial and final ankle weight (WGT, kg) lifted. Electromyographic variables included: fiber density (FD), jitter (MCD), and blocking (BLK) from single fiber assessment and median macro amplitude (MACRO). Serum creatine kinase (CK) was also measured initially and at 4-week intervals throughout the study.

RESULTS: The following variables significantly (p < .05) increased: WGT by 47%, ISOKPT, 15%, ISOKTW, 15%; MVC, 36%; EDUR, 21%; TTI, 18%. The following variables did not significantly (p > .05) change: FD, MCD, BLK, MACRO, and CK.

CONCLUSIONS: This home exercise program significantly increased strength, endurance, and TTI without apparently adversely affecting the motor units or the muscle, as the EMG and CK variables did not change.

A case of post-poliomyelitis muscular atrophy with cranial nerve signs and widespread muscular atrophy of extremities

Author(s): Hoshino S, Hayashi A, Ohkoshi N, Mizusawa H, Shoji S
Affiliation(s): Department of Neurology, University of Tsukuba

Publication(s): Rinsho Shinkeigaku (Clinical Neurology)
Citation: Rinsho Shinkeigaku. 1997 May; 37(5):407-9

Here we report a case of a 56-year-old male with post-poliomyelitis muscular atrophy (PPMA), who presented with cranial nerve signs and widespread atrophy of the extremities. He had suffered from poliomyelitis at the age of 2 years. After recovery from the acute stage, the paralysis remained in his left arm. He noticed muscle weakness of the right upper and lower extremities at the age of 45 years and the muscle atrophy progressed to his arms, hip and thigh at the age of 55 years. Neurological examination revealed muscle atrophy of the neck and disturbance of left V, VIII, IX, X and bilateral XI cranial nerves. We diagnosed this case as PPMA from his history and electromyographic and muscle biopsy findings which suggested chronic denervation. Among the 21 PPMA cases in the past in which the acute poliomyelitis had resulted in paralysis of the only one limb, ours was the only case that had muscle atrophy of all the limbs. Cranial nerve involvement is known to occur in acute poliomyelitis; therefore, there is a possibility that the involvement of the cranial nerves in our case might be a delayed progressive symptoms.

This paper is in Japanese

Reduction in thigh muscle cross-sectional area and strength in a 4-year follow-up in late polio

Author(s): Grimby G, Kvist H, Grangård U
Affiliation(s): Department of Rehabilitation Medicine, University of Göteborg, Sahlgrenska University Hospital, Sweden

Publication(s): Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 1996 Oct; 77(10):1044-8

OBJECTIVE: To study changes in cross-sectional thigh muscle area and muscle strength in late polio subjects over a 4-year period.

DESIGN: Longitudinal study of a cohort of polio survivors, comparing subjects who acknowledge (unstable) with those who do not acknowledge (stable) new muscle weakness.

SETTING: University hospital.

SUBJECTS: Eighteen subjects (6 men, 12 women) with polio-myelitis sequelae (39 to 46 years of age) were studied on two occasions 4 years apart; the first examination was 37 to 44 years after onset of polio. Subjects were recruited through hospital registers, newspaper advertisement, and a patient organization.

OUTCOME MEASUREMENTS: Thigh muscle and intermuscular and intramuscular adipose tissue (AT) cross-sectional areas were measured by computed tomography. Isometric muscle strength for knee extension and flexion was measured using a Kin-Com dynamometer.

RESULTS: Cross-sectional muscle area decreased on average 1.3 +/- 3.6 cm2 (1.4%, p < .05); the intermuscular and intramuscular AT area increased 1.8 +/- 3.4 cm2 (12.1%, p < .05). When divided by legs in which subjects reported (unstable) or did not report (unstable) or did not report (stable) increased muscle weakness, unstable legs showed significant reduction (p < .05) in muscle area, whereas stable legs did not. Estimated total thigh muscle strength decreased 7.8% +/- 2.9% (p < .01), with a significant (p < .001) reduction in unstable legs (13.4% +/- 4.3%) but not in stable legs. The reduction in strength appears to be greater than the reduction in cross-sectional muscle area, but there is still a significant correlation (r = .44, p < .05).

CONCLUSION: The present results demonstrate not only progress of muscle weakness, but also of muscle atrophy in postpolio subjects.

Living with the late effects of disability: A five-year follow-up survey of coping among post-polio survivors

Author(s): Westbrook M, McIlwain D
Affiliation(s): Faculty of Health Sciences, The University of Sydney

Publication(s): Australian Occupational Therapy Journal
Citation: Aust Occup Ther J. 1996 June;43(2):60-71

A follow-up survey of 176 people aged 33–77 yrs with postpolio syndrome found that while they were experiencing significantly less anxiety, uncertainty, depression, and helplessness concerning their increasing disability than 5 yrs ago, their feelings of anger persisted. Relationships were found between coping styles, breadth of coping attempted, and emotions experienced. Specifically, a coping style of focusing on symptoms while attempting to maintain previous activity levels characterized people who felt more helpless, depressed, and angry. Those who coped by accommodating to their symptoms had adopted more strategies. Coping strategies involving lifestyle and personal changes were rated as more effective than many treatments and interpersonal strategies. Findings highlight the need for health practitioners to tap into clients' knowledge when designing and evaluating appropriate rehabilitation programs.

The full paper is available from Polio Australia for private study purposes

Dynamic electromyography and muscle biopsy changes in a 4-year follow-up: study of patients with a history of polio

Author(s): Stålberg E, Grimby G
Affiliation(s): Department of Clinical Neurophysiology, University Hospital, Uppsala, Sweden

Publication(s): Muscle & Nerve
Citation: Muscle Nerve. 1995 Jul;18(7):699-707

Eighteen patients who had had polio 29-56 years prior to the first investigation were studied on two occasions, 4 years apart. Isokinetic and isometric strength measurements and Macro EMG were performed in 28 legs. Muscle biopsy specimens were obtained on both occasions from 11 legs. On average the muscle strength was 56% of control values at the first examination, and decreased by another 8% during the observation period. The muscle fiber area was increased compared to that of controls and did not change significantly. Macro EMG, comprising muscle fiber area and number of muscle fibers, and/or single fiber EMG showed clear signs of reinnervation in all legs. The motor units at the first examination were increased 11-fold, on average, compared with age-matched control values. During the observation period, reinnervation continued and the size of motor units increased by another 56% as a result of ongoing denervation, that is, loss of neurons. This compensation was particularly pronounced in patients with stable conditions. The parameters studied did not reveal any definite pattern predicting future development of new muscle weakness in individual subjects.

Fractures in an aging population of poliomyelitis survivors: a community-based study in Olmsted County, Minnesota

Author(s): Goerss JB, Atkinson EJ, Windebank AJ, O'Fallon WM, Melton LJ 3rd
Affiliation(s): Department of Medical Genetics, Mayo Clinic Rochester, Minnesota 55905

Publication(s): Mayo Clinic Proceedings
Citation: Mayo Clin Proc. 1994 Apr; 69(4):333-9

OBJECTIVE: To determine the incidence of fractures in a cohort of survivors of paralytic poliomyelitis (“polio”).

DESIGN: We conducted a population-based retrospective cohort study of residents of Olmsted County, Minnesota, who had an initial diagnosis of polio between 1935 and 1959 and survived the acute illness.

MATERIALS AND METHODS: A comprehensive medical records-linkage system was used to retrieve all diagnoses of poliomyelitis in residents of the county for the specified 25-year period. For the 277 study subjects, the medical records were searched for the occurrence of fracture from the time of initial diagnosis of polio until death or the most recent clinical contact. All fractures were classified on the basis of type of associated injury and skeletal site of involvement. The influence of polio on the incidence of fractures was evaluated by estimating the cumulative incidence of new fractures after the diagnosis of polio and the standardized morbidity ratio. The relative influence of various factors on the risk of fracture was determined.

RESULTS: Of the 277 Olmsted County residents with polio, 87 experienced 161 fractures, and the estimated cumulative incidence of any fracture after 40 years was 48%. The cumulative incidence of any limb fracture was 41% and was less than the expected value of 44% (P = 0.001). Only the risk of distal femoral and proximal humeral fractures was significantly higher than that among Olmsted County residents in general. The increased risk seemed to be associated with weakness and disuse of the involved limbs rather than with generalized osteoporosis. Theoretically, a greater capacity for bone remodeling in response to changes in muscle use might have protected patients with polio in childhood, but such persons were as likely to sustain a fracture as those with adult-onset poliomyelitis.

CONCLUSION: These data indicate that survivors of paralytic poliomyelitis do not have an unusual risk of fracture except in affected limbs.

Coping with the late effects: differences between depressed and nondepressed polio survivors

Author(s): Tate D, Kirsch N, Maynard F, Peterson C, Forchheimer M, Roller A, Hansen N
Affiliation(s): Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor

Publication(s): American Journal of Physical Medicine & Rehabilitation
Citation: Am J Phys Med Rehabil. 1994 Feb;73(1):27-35

This study examined differences between depressed and nondepressed individuals with a history of paralytic poliomyelitis in terms of demographics, health status and coping strategies. The prevalence of distress and depression in this group of 116 polio survivors was determined. Subjects completed the Brief Symptom Inventory, the Coping with Disability Inventory and a questionnaire concerning their polio histories and self-perceptions of health. Medical assessments were performed by physicians. Only 15.8% of the sample had scores indicating depression and elevated distress. Depressed/distressed polio survivors were more likely to: be living alone, be experiencing further health status deterioration, seek professional help, view their health as poor, report greater pain, be less satisfied with their occupational status and their lives in general and exhibit poorer coping outcome behaviors in relation to their disability. Three factors in coping with the late effects of polio were identified through a factor analysis of the Coping with Disability Inventory: positive self-acceptance, information seeking/sharing about the disability and social activism. Differences between depressed/distressed and other polio survivors were found across these three factors, with depressed/distressed subjects having significantly lower coping scores. These and other results are discussed.

The full paper is available from Polio Australia for private study purposes

Prevalence and associated features of depression and psychological distress in polio survivors

Author(s): Tate DG, Forchheimer M, Kirsch N, Maynard F, Roller A
Affiliation(s): Department of Physical Medicine and Rehabilitation, University of Michigan Medical Center, Ann Arbor

Publication(s): Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 1993 Oct;74(10):1056-60

This cross-sectional study examines the prevalence of psychological distress and depression among 116 polio survivors. It investigates demographic, medical, and coping differences between subjects with (n = 17) and without (n = 99) these symptoms. Subjects were administered the Brief Symptom Inventory (BSI), the Coping with Disability Inventory (CDI), and a questionnaire about their polio histories. The BSI provided measures of psychological distress and depression that defined the subgroups. The CDI assessed coping behaviors. BSI scores for the overall sample were within the normal range indicating no major distress, depression, nor elevated somatic complaints. Several significant differences were found between the two subgroups. On average, depressed/distressed subjects reported an increase in pain (p < .01) and further deterioration of their medical status since the time of their physical best subsequent to the onset of polio (p < .01). They consistently rated their health as poorer than did nondepressed/nondistressed subjects (p < .001). They also reported less satisfaction with life and their occupational status (p < .001) and displayed poorer coping behaviors combined (p < .001). Selected variables such as life satisfaction, pain, decrease in activity, and current living situation accounted for 51% of the variance when predicting distress and depression among this group of polio survivors.

The full paper is available from Polio Australia for private study purposes

Epidemiology of the post-polio syndrome

Author(s): Ramlow J, Alexander M, LaPorte R, Kaufmann C, Kuller L
Affiliation(s): Department of Biostatistics, Graduate School of Public Health, University of Pittsburgh, PA

Publication(s): American Journal of Epidemiology
Citation: Am J Epidemiol. 1992 Oct 1;136(7):769-86

A late-onset syndrome, consisting of muscle weakness, muscle pain, and unaccustomed fatigue, has been reported with increasing frequency among former poliomyelitis patients. A population-based cohort of poliomyelitis patients from Allegheny County, Pennsylvania, was traced and surveyed to estimate the prevalence and incidence and to identify determinants of the post-polio syndrome. A questionnaire validated in clinical examinations of 40 cohort members was used in the survey. The prevalence of the post-polio syndrome was 28.5% of all paralytic cases (95% confidence interval 24.4-32.6). The risk of post-polio syndrome was significantly higher among patients who sustained substantial permanent impairment after polio and among females. The incidence did not vary with age at acute onset, acute severity, or level of physical activity after recovery. The strongest determinant of post-polio syndrome onset was the length of the interval following the acute illness, with incidence peaking at 30-34 years. Of all cases of post-polio syndrome, 79% reported no major change in impairment status since onset. This study demonstrates that poliomyelitis patients are not equally susceptible to post-polio syndrome within the interval of 30-40 years after the original illness. For syndrome cases, the onset was associated with new neuromuscular symptoms and functional changes but not with major new impairment.

Incidence and nature of dysphagia in polio survivors

Author(s): Coelho CA, Ferranti R
Affiliation(s): Department of Communication Disorders, Gaylord Hospital, Wallingford, CT 06492

Publication(s): Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 1991 Dec; 72(13):1071-5

Questionnaires pertaining to swallowing function were mailed to 220 members of postpolio support groups in Connecticut. Of the 109 responses, 80 individuals reported having no difficulty with swallowing, while 29 reported having either intermittent or consistent swallowing problems. Twenty-one of the 29 were seen for videofluoroscopic swallowing studies and pulmonary function testing. The swallowing studies showed that 43% of these individuals had difficulty with bolus control, 19% with delayed swallow response, and 81% with decreased pharyngeal transit. Although none of these individuals were observed to aspirate, two were judged to be at significant risk. Incidence of dysphagia within the group of polio survivors was estimated to be approximately 18%. Seventeen of the 20 postpolio subjects with dysphagia also demonstrated decreased breathing capacity. Although moderately to severely depressed values in the pulmonary function measures accompanied moderate dysphagia in certain postpolio individuals, reduced values in these same measures were also present in individuals with minimal swallowing dysfunction. Therefore, although impaired breathing may complicate swallowing dysfunction and vice versa, it does not appear that one can be predicated from the other. Management of dysphagia in postpolio individuals is discussed.

Neuromuscular function in polio survivors

Author(s): Agre JC, Rodriquez AA
Affiliation(s): Department of Rehabilitation Medicine, University of Wisconsin - Madison Medical School 53792

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Dec; 14(12):1343-7

Although there is no documented, objective evidence that symptomatic post-polio subjects are rapidly losing strength, they have a number of neuromuscular deficits related to a more severe poliomyelitis illness that may explain why they complain of problems with strength, endurance, and local muscle fatigue. Symptomatic post-polio subjects were hospitalized longer during the acute poliomyelitis, recovered more slowly, and had electromyographic evidence of greater loss of anterior horn cells. Additionally, recent assessment demonstrated that they were weaker, had a reduced work capacity, and recovered strength less readily after activity in the quadriceps muscles as compared to asymptomatic subjects. Of great clinical importance, rating of perceived exertion in the muscle during exercise was the same in symptomatic and asymptomatic post-polio and control subjects, indicating that symptomatic subjects have a mechanism to monitor local muscle fatigue that could be used to avoid exhaustion. A study of pacing (interspersing activity with rest breaks) showed that symptomatic subjects had less local muscle fatigue and greater strength recovery when they paced their activity than when they worked at a constant rate to exhaustion. We recommend that post-polio individuals pace their daily activity to avoid excessive fatigue.

Electrophysiology and electrodiagnosis of the post-polio motor unit

Author(s): Trojan DA, Gendron D, Cashman NR
Affiliation(s): Department of Neurology, Montreal Neurological Institute and Hospital, Quebec, Canada

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Dec; 14(12):1353-61

Post-poliomyelitis syndrome refers to new symptoms that may occur years after recovery from poliomyelitis. The most common of these symptoms are new weakness, fatigue, and pain. This article describes electrodiagnostic studies--conventional electromyography (EMG), single fiber electromyography (SFEMG), and macroelectromyography (macro-EMG) – that have provided information on the post-polio motor unit and on the possible etiology of some post-polio syndrome symptoms. Muscular fatigue, and indirectly, general fatigue, may be due to neuromuscular junction transmission defects in some post-polio individuals, as suggested by reduction of the compound motor action potentials on repetitive stimulation, and increased jitter and blocking on SFEMG. Progressive weakness and atrophy in post-polio syndrome is probably due to a distal degeneration of post-polio motor units with resultant irreversible muscle fiber denervation. Electrodiagnostic evidence of ongoing denervation includes fibrillation and fasciculation potentials on conventional EMG, increased jitter and blocking on SFEMG, and smaller macro-EMG amplitudes in newly weakened post-polio muscles. However, even though electrodiagnostic studies have provided insight into the possible causes of some post-polio syndrome symptoms, no specific electrodiagnostic test for the syndrome is currently available.

A rat model of the post-polio motor unit

Author(s): Pachter BR, Eberstein A
Affiliation(s): Department of Rehabilitation Medicine, New York University Medical Center, NY 10016

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Dec; 14(12):1367-73

We examined the long-term effects of muscle usage on a rat model of the post-polio motor unit. Isometric tensions, type I and type II muscle fiber areas, the incidence of collateral sprouting, and motor endplate morphology were examined following 1, 3, 6, and 9 months of partial denervation in rat plantaris muscle. Full morphologic and functional stability of the expanded motor units occurred at 6 months post-partial denervation. Fiber hypertrophy was observed, possibly the result of compensatory work hypertrophy due to muscle overuse. Following 9 months of partial denervation and muscle overuse, the twitch and tetanic tensions and type I and type II muscle fiber areas were significantly reduced as compared to sham controls; angulated myofibers and group atrophy also were seen. The percent collateral sprouting, the number of terminal branches per endplate, and the endplate area were all increased, possibly a compensatory response to a decreased synthesis of neurotrophic factor(s) and/or transmitter-related components. These aging-like changes seem to occur earlier in chronically stressed, overenlarged, and overworked motor units.

The cultural context of polio biographies

Author(s): Scheer J, Luborsky ML
Affiliation(s): National Rehabilitation Hospital Research Center, Washington, DC 20010

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1173-81

Cultural contexts influence the ways individuals interpret and experience functional losses associated with post-polio sequelae. Using in-depth multiple interview case studies from two National Institute on Aging projects, the concept of “biographies” is presented to place the individuals’ polio-related experiences within the context of their lives. Two major cultural contexts shape the construction of polio biographies: normative life course expectations and developmental tasks; and traditions associated with polio recovery and rehabilitation. The authors identify key dimensions of personal concern among polio survivors that can be used as entrance points for effective clinical intervention and to promote treatment compliance.

The psychology of polio as prelude to post-polio sequelae: behavior modification and psychotherapy

Author(s): Bruno RL, Frick NM
Affiliation(s): Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation, East Orange, NJ

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1185-93

Even as the physical causes and treatments for post-polio sequelae (PPS) are being identified, psychological symptoms – chronic stress, anxiety, depression, and compulsive, Type A behavior – are becoming evident in polio survivors. Importantly, these symptoms are not only causing marked distress but are preventing patients from making the lifestyle changes necessary to treat their PPS. Neither clinicians nor polio survivors have paid sufficient attention to the acute polio experience, its conditioning of life-long patterns of behavior, its relationship to the development of PPS, and its effect on the ability of individuals to cope with and treat their new symptoms. We describe the acute polio and post-polio experiences on the basis of patient histories, relate the experience of polio to the development of compulsive, Type A behavior, link these behaviors to the physical and psychological symptoms reported in the National Post-Polio Surveys, and present a multimodal behavioral approach to treatment.

The full text of this paper (as revised 7 February 2000) has been generously made available by Dr Bruno
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Effect of treatment and noncompliance on post-polio sequelae

Author(s): Peach PE, Olejnik S
Affiliation(s): Roosevelt Warm Springs Institute for Rehabilitation, Georgia 31830

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1199-203

In this study of 77 patients with post-polio sequelae (PPS), symptoms and manual test scores on initial evaluation were compared with those at subsequent follow-up evaluations. Patients were divided into three groups based on the degree to which they had complied with clinically recommended interventions: compliers, partial compliers, and noncompliers. At the end of the follow-up period (2.2 +/- 1.2 years), the mean muscle function scores of the entire study group had declined -1.5%, which represented a decline of -0.7% annually. On follow-up evaluations, the complier group had realized an improvement or resolution of post-polio symptoms, and also an improvement in muscle function of +0.6% annually. The partial complier group had realized either no improvement, or improvement in post-polio symptoms, but showed a further decline in muscle function of -3.0%, or an annual decline of -1.3%. The noncomplier group showed either no change, or a worsening of post-polio symptoms, and also showed a further decline in muscle function of -4.1%, which represented an annual decline of -2.0%.

Activity and post-polio fatigue

Author(s): Packer TL, Martins I, Krefting L, Brouwer B
Affiliation(s): Division of Occupational Therapy, School of Rehabilitation Therapy, Queen's University, Kingston, Ontario, Canada

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1223-6

Fatigue was studied in 12 subjects with post-polio sequelae (PPS). Results of the Fatigue Severity Scale (FSS) demonstrated a mean score of 4.8 +/- 1.6 (non-disabled scores = 2.3 +/- 0.7). The Human Activity Profile (HAP) was not sensitive enough to measure fatigue. Fifty percent of subjects scored below the first percentile based on age and sex matched norms. The Activity Record (ACTRE) results revealed that subjects spent 5% of their time resting and 1% in planning or preparation activities. Fatigue peaked in the late morning or early afternoon and was relieved by rest periods. Use of energy conservation and work simplification skills along with frequent rest periods was suggested as a possible method for managing PPS fatigue.

Energy conservation, occupational therapy, and the treatment of post-polio sequelae

Author(s): Young GR
Affiliation(s): Department of Occupational Therapy, Kaiser-Permanente Medical Center, Downey, California 90242

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1233-9

Individuals experiencing post-polio sequelae (PPS) are usually advised to make significant lifestyle changes to lessen symptoms and prevent further decline in function. These individuals have spent most of their lives equating success with over-achievement and find it difficult to implement such recommendations. As specialists in energy conservation and work simplification, occupational therapists increasingly are being called on to evaluate and treat these patients. Over the past 2 years, an occupational therapy educational program has been developed to educate patients about their condition and about ways to implement lifestyle changes while preserving the ability to do valued activities. This article describes the components of a thorough occupational therapy evaluation and the design and functional outcomes of a successful occupational therapy educational program to treat PPS.

Effect of modified aerobic training on movement energetics in polio survivors

Author(s): Dean E, Ross J
Affiliation(s): School of Rehabilitation Medicine, University of British Columbia, Vancouver, Canada

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1243-6

Given that individuals with disabilities may be unable to achieve maximal oxygen uptake in an exercise test and that maximal exercise testing may cause increased fatigue, pain, and muscle weakness, we examined the role of submaximal exercise testing and training based on objective as well as subjective parameters in polio survivors. Experimental (N = 7) and control subjects (N = 13) were tested before and after a 6-week period. The experimental subjects participated in a 6-week exercise training program for 30 to 40 minutes, three times a week. The program consisted of treadmill walking at 55% to 70% of age-predicted maximum heart rates; however, exercise intensity was modified to minimize discomfort/pain and fatigue. Neither objective nor subjective exercise responses were significantly different in the control group over the 6 weeks. No change was observed in cardiorespiratory conditioning in the experimental group. However, movement economy, which is related to the energy cost of walking, was significantly improved; and walking duration was significantly increased at the end of training. Modified aerobic training may have a role in enhancing endurance and reducing fatigue during activities of daily living in polio survivors.

The effects of long-term non-fatiguing resistance exercise in subjects with post-polio syndrome

Author(s): Fillyaw MJ, Badger GJ, Goodwin GD, Bradley WG, Fries TJ, Shukla A
Affiliation(s): Department of Physical Therapy, University of Vermont, Burlington

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1253-6

Measures of torque were used to evaluate changes in muscle strength and endurance in 17 patients with post-polio syndrome who did prescribed resistance exercise for up to 2 years. Exercise compliance averaged 75%, with 16 subjects increasing the weight lifted in training. Maximum torque was significantly increased in the exercised muscle compared to the control muscle; no difference was seen in muscle endurance. Individuals with post-polio syndrome can increase muscle strength by doing non-fatiguing resistance exercise, but they should undergo quantitative testing of muscle strength a minimum of every 3 months to guard against overwork weakness.

Post-polio fatigue: a 31P magnetic resonance spectroscopy investigation

Author(s): Thompson RT, Barton PM, Marsh GD, Cameron MG, Gravelle DG, Hsieh JT, Hayes KC, Driedger AA
Affiliation(s): Department of Nuclear Medicine, St. Joseph's Health Center, London, Ontario, Canada

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1263-7

Changes in high energy phosphates (HEP) and intramuscular pH during exercise were measured in 17 patients with post-polio fatigue and in 28 healthy controls using 31P magnetic resonance spectroscopy (MRS). Subjects performed a dynamic hand grip exercise at low and high intensity. Mean changes in the HEP and pH showed no significant differences between the groups, although the post-polio group's response was highly variable. Six patients showed evidence of a lower lactate accumulation during the high intensity exercise when compared with controls. These data suggest that the whole body fatigue experienced by polio survivors is not related to any systemic metabolic abnormality.

Polioencephalitis, stress, and the etiology of post-polio sequelae

Author(s): Bruno RL, Frick NM, Cohen J
Affiliation(s): Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation, East Orange, NJ 07018

Publication(s): Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1269-76

Post-mortem neurohistopathologies that document polio virus-induced lesions in reticular formation and hypothalamic, thalamic, peptidergic, and monoaminergic neurons in the brain are reviewed from 158 individuals who contracted polio before 1950. This polioencephalitis was found to occur in every case of poliomyelitis, even those without evidence of damage to spinal motor neurons. These findings, in combination with data from the 1990 National Post-Polio Survey and new magnetic resonance imaging studies documenting post-encephalitis-like lesions in the brains of polio survivors, are used to present two hypotheses: 1) polioencephalitic damage to aging reticular activating system and monoaminergic neurons is responsible for post-polio fatigue, and 2) polioencephalitic damage to enkephalin-producing neurons is responsible for hypersensitivity to pain in polio survivors. In addition, the antimetabolic action of glucocorticoids on polio-damaged, metabolically vulnerable neurons may be responsible for the fatigue and muscle weakness reported by polio survivors during emotional stress.

The full text of this paper has been generously made available by Dr Bruno
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Late effects of polio: critical review of the literature on neuromuscular function

Author(s): Agre JC, Rodríquez AA, Tafel JA
Affiliation(s): University of Wisconsin - Madison Medical School

Publication(s): Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 1991 Oct; 72(11):923-31

Many individuals who have had poliomyelitis are now complaining of several new problems attributed to their former illness including muscle atrophy; fatigue; progressive weakness; and muscle, back, and joint pain. This paper critically examines the literature regarding the neuromuscular effects of poliomyelitis. Weakness resulting from poliomyelitis was due to destruction of anterior horn cells. After the illness, muscle strength was partially recovered as a result of several physiologic adaptive mechanisms including terminal sprouting and reinnervation, myofiber hypertrophy, and, possibly, myofiber type transformation. Several pathophysiologic and functional etiologies have been proposed for late neuromuscular deterioration, but none has been proven. In fact, to date, there is no objective evidence documenting progressive loss of strength in polio survivors. Studies attempting to differentiate polio survivors with and without symptoms of deterioration have resulted in conflicting results; however, it appears reasonable to conclude that symptomatic postpolio subjects had a more severe illness with greater loss of neuromuscular function. Exercise may be helpful for many postpolio patients, but the prescription must be tailored to the individual to avoid problems of overuse or excessive fatigue.

Intrathecal immune response in patients with the post-polio syndrome

Author(s): Sharief MK, Hentges R, Ciardi M
Affiliation(s): Department of Clinical Neurochemistry, National Hospital for Neurology and Neurosurgery, London, United Kingdom

Publication(s): The New England Journal of Medicine
Citation: N Engl J Med. 1991 Sep 12;325(11):749-55

BACKGROUND: The syndrome of progressive muscular atrophy decades after acute paralytic poliomyelitis (post-polio syndrome) is not well understood. The theory that physiologic changes and aging cause the new weakness does not explain the immunologic abnormalities reported in some patients. An alternative explanation is persistent or recurrent poliovirus infection.

METHODS: We assessed the intrathecal antibody response to poliovirus and intrathecal production of interleukin-2 and soluble interleukin-2 receptors in 36 patients with the post-polio syndrome and 67 controls (including 13 who had had poliomyelitis but had no new symptoms and 18 with amyotrophic lateral sclerosis). Intrathecal antibody responses to measles, mumps, herpes simplex, and varicella zoster viruses were also determined.

RESULTS: Oligoclonal IgM bands specific to poliovirus were detected in the cerebrospinal fluid of 21 of the 36 patients with the post-polio syndrome (58 percent) but in none of the control group (P less than 0.0001). In quantitative studies there was evidence of increased intrathecal synthesis of IgM antibodies to poliovirus only among the patients with the post-polio syndrome; there was no increased synthesis of IgM to measles, mumps, herpes simplex, or varicella zoster viruses. The patients with post-polio syndrome had significantly higher mean (+/- SD) (cerebrospinal fluid levels of interleukin-2 and soluble interleukin-2 receptors than the controls (8.1 +/- 5.3 vs. 1.4 +/- 0.8 U per milliliter and 159.6 +/- 102.9 vs. 10.7 +/- 6.2 U per milliliter, respectively). The intrathecal synthesis of IgM antibodies to poliovirus correlated with the cerebrospinal fluid concentrations of interleukin-2 (P less than 0.0005) and soluble interleukin-2 receptors (P less than 0.001).

CONCLUSIONS: An intrathecal immune response against poliovirus is present in many patients with the post-polio syndrome. In some of these patients the recrudescence of muscle weakness may be caused by persistent or recurrent infection of neural cells with the poliovirus.

See also comments in:
N Engl J Med. 1992 Feb 27;326(9):641; author reply 642
N Engl J Med. 1992 Feb 27;326(9):640-1; author reply 642
N Engl J Med. 1992 Feb 27;326(9):641; author reply 642

Recognizing typical coping styles of polio survivors can improve re-rehabilitation – A commentary

Author(s): Maynard FM, Roller S
Affiliation(s): Department of Physical Medicine and Rehabilitation, University of Michigan Medical Center, Ann Arbor 48109-0491

Publication(s): American Journal of Physical Medicine & Rehabilitation
Citation: Am J Phys Med Rehabil. 1991 Apr;70(2):70-2

This paper does not have an abstract

The full paper is available from Polio Australia for private study purposes

Evaluation, treatment, and follow-up results of post polio patients with dysphagia

Author(s): Silbergleit AK, Waring WP, Sullivan MJ, Maynard FM
Affiliation(s): Department of Physical Medicine, University of Michigan, Ann Arbor

Publication(s): Otolaryngology Head and Neck Surgery
Citation: Otolaryngol Head Neck Surg. 1991 Mar;104(3):333-8

Twenty consecutive patients were evaluated for reports of dysphagia from post-polio clinics. Only half the patients reported a history of swallowing problems at the time of their acute poliomyelitis. Each patient received a videofluorographic evaluation of the oral and pharyngeal phases of swallowing, and then was provided with recommendations to improve swallowing skills. A follow-up questionnaire was sent to all patients. The respondents had an average interval of 12 months since the initial evaluation. Of the 18 patients responding to the questionnaire, 14 (77%) reported regular use of the swallowing suggestions. Comparison of pre-evaluation results to followup of the 18 respondents yielded a statistically significant decline in the frequency of choking (p = 0.0156) and food sticking in the throat (p = 0.0195). We conclude that a dysphagia program can result in significant improvement of the swallowing symptoms reported with the post-polio population.

Neuromuscular function: comparison of symptomatic and asymptomatic polio subjects to control subjects

Author(s): Agre JC, Rodriquez AA
Affiliation(s): Department of Rehabilitation Medicine, University of Wisconsin - Madison Medical School 53792

Publication(s): Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 1990 Jul; 71(8):545-51

The purpose of this study was to determine if there were any differences between symptomatic and asymptomatic polio survivors by history of acute poliomyelitis illness, electromyographic evidence of terminal motor unit reorganization, and neuromuscular function of the quadriceps femoris muscle. Thirty-four symptomatic postpolio subjects, 16 asymptomatic postpolio subjects, and 41 controls were studied. A questionnaire assessed polio history. Peak knee extension torque was measured isokinetically and isometrically. Endurance (time to exhaustion) was measured at 40% of maximal isometric torque. Work capacity was determined as the product of torque and duration. Recovery of isometric strength was measured at regular intervals for ten minutes after exhaustion. Quantitative electromyography was also performed on the quadriceps to determine motor unit action potential duration and amplitude. It was found that symptomatic subjects had evidence of more severe original polio involvement by history (documented electromyographically), were weaker and capable of performing less work than asymptomatic subjects, and recovered strength less readily than controls.

Electromyographic and morphological functional compensation in late poliomyelitis

Author(s): Einarsson G, Grimby G, Stålberg E
Affiliation(s): Department of Rehabilitation Medicine, Gothenburg University, Sweden

Publication(s): Muscle & Nerve
Citation: Muscle Nerve. 1990 Feb; 13(2):165-71

Patients with prior poliomyelitis may experience muscle function deterioration decades after onset of disease. The present study is aimed at describing electromyographic and morphometric evidence of muscular compensation and of on-going muscular instability. Ten subjects 42-62 years of age with onset of polio 25-52 years earlier were studied with macro EMG, single-fiber EMG (SFEMG), muscle strength measurement, and morphometrical analysis of muscle biopsies from the vastus lateralis muscle. SFEMG revealed increased fiber density (FD) and large macro-MUP potentials indicating pronounced reinnervation as compensation to loss of motor neurons. From electrophysiological data of motor unit size, morphometric measures of fiber size, and muscle strength data, the minimal degree of motor neuron loss was estimated to be greater than 70%.

Cardiorespiratory Responses to Aerobic Training by Patients With Postpoliomyelitis Sequelae

Author(s): Jones DR, Speier J, Canine K, Owen R, Stull GA
Affiliation(s): Sister Kenny Institute, Minneapolis, Minn (Mr Jones and Drs Speier, Canine, and Owen) School of Health Related Professions, State University of New York at Buffalo (Dr Stull)

Publication(s): The Journal of the American Medical Association
Citation: JAMA. 1989 Jun 9;261(22):3255-58

We examined the cardiorespiratory responses of 16 patients with postpoliomyelitis sequelae to a 16-week aerobic exercise program. The patients exercised at 70% of maximal heart rate. Dependent variables were resting and maximal heart rates, systolic and diastolic blood pressures, maximum oxygen consumption, maximum carbon dioxide consumption, respiratory quotient, and maximum expired volume per unit time. The exercise group was superior to the control group in watts, exercise time, maximum expired volume per unit time, and maximum oxygen consumption. No untoward events or loss of leg strength occurred as a result of the exercise regimen. We conclude that the aerobic training program employed in this study is a safe, short-term procedure and that patients with postpolio sequelae respond to training in a manner similar to healthy adults.

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The postpolio syndrome – An overuse phenomenon

Author(s): Perry J, Barnes G, Gronley JK
Affiliation(s): Rancho Los Amigos Medical Center, Downey, CA 90242

Publication(s): Clinical Orthopedics and Related Research
Citation: Clin Orthop Relat Res. 1988 Aug;(233):145-62

Persons with good recovery of function following their initial poliomyelitis are now, more than 30 years later, experiencing new weakness, fatigue, and muscle pain. The likelihood of muscle overuse being the cause of this late functional loss was investigated by dynamic electromyography (EMG) and foot-switch stride analysis in 34 symptomatic patients. Manual testing grouped the muscles, with strong (S) encompassing Grades Good (G) and Normal (N) while weak (W) included Fair plus (F+) to zero (0). After testing quadriceps and calf strength, the patients fell into one of four classes: strong quadriceps and calf (SQ/SC), strong quadriceps and weak calf (SQ/WC), weak quadriceps and strong calf (WQ/SC), or combined weak quadriceps and calf (WQ/WC). Quantified EMG (normalized by the manual muscle test EMG) defined the mean duration and intensity of the quadriceps, soleus, lower gluteus maximus, and long head of the biceps femoris during walking. Overuse was defined as values greater than the laboratory normal (mean + 1 SD). Each muscle exhibited instances of overuse, normalcy, and sparing. The biceps femoris was the only muscle with dominant overuse (82%). Quadriceps overuse was next in frequency (53%). Soleus activity infrequently exceeded normal function (34%), but this still represented more than twice the intensity and duration of the other muscles. Gluteus maximus action was also seldom excessive (34%). The patients averaged two muscles with excessive use during walking. Gait velocity of the SQ/SC strong group was highest (71% of normal) while the three categories that included weak muscles had walking speeds in the range of 50% of normal. The finding of muscle overuse during a single free-speed walking test that does not attain normal velocity supports the concept of muscle overuse being the cause of the patient's dysfunction.

Functional recovery – A major risk factor for the development of postpoliomyelitis muscular atrophy

Author(s): Klingman J, Chui H, Corgiat M, Perry J
Affiliation(s): Department of Neurology, University of Southern California School of Medicine, Los Angeles

Publication(s): Archives of Neurology
Citation: Arch Neurol. 1988 Jun;45(6):645-7

A retrospective study was undertaken to identify potential risk factors for the development of progressive postpoliomyelitis muscular atrophy (PPMA). Patients with PPMA (n = 57) were compared with patients with a history of poliomyelitis but without a history of progressive weakness (n = 49). Patients who later developed PPMA had histories of more widespread acute paralysis, but relatively greater functional recovery. They were less disabled, and reported higher recent activity levels. Seventy-nine percent of the total variance between the PPMA and control groups could be accounted for by recovery alone (ie, severity minus disability). Functional recovery is generally attributed to reinnervation of sarcomeres by collateral sprouting from surviving lower motor neurons. Since degree of recovery predicts the risk of developing PPMA, our findings suggest that enlarged motor units may carry an increased susceptibility for dysfunction and/or degeneration.

Late denervation in patients with antecedent paralytic poliomyelitis

Author(s): Cashman NR, Maselli R, Wollmann RL, Roos R, Simon R, Antel JP
Affiliation(s): Not stated

Publication(s): The New England Journal of Medicine
Citation: N Engl J Med. 1987 Jul 2; 317(1):7-12

The development of new weakness, fatigue, and pain decades after acute paralytic poliomyelitis is a recognized syndrome. We conducted a controlled study of this syndrome by analyzing clinical, electromyographic, and muscle-biopsy features in 18 patients with a history of poliomyelitis--13 reporting 1 to 20 years of new weakness and 5 without new symptoms. The patients with new weakness also reported new muscle atrophy (9 of 13) and fatigue (10 of 13), symptoms not reported by the controls. The age at the time of acute poliomyelitis, severity of poliomyelitis, residual disability, number of years since acute poliomyelitis, and age at the time of study were comparable in the weakening and control groups. Evidence of remote denervation consistent with antecedent poliomyelitis was demonstrated in all patients by electromyography or muscle biopsy or both. In addition, active denervation (as evidenced by spontaneous activity on conventional electromyography, increased jitter on single-fiber electromyography, or atrophic myofibers) was found in 12 patients in the weakening group and in all 5 controls. Immunohistochemical detection of myofibers expressing the neural-cell adhesion molecule corroborated ongoing denervation in both patient groups. When muscle data from both groups were pooled, correlations were observed between the extent of past reinnervation and the degree of ongoing motor-unit instability. We conclude that the extensive reinnervation of denervated muscle that occurs in paralytic poliomyelitis may be followed by late denervation of the previously reinnervated muscle fibers. Electromyographic and muscle-biopsy evidence of ongoing denervation does not distinguish between stable patients with prior paralytic poliomyelitis and those with new weakness.

Neurological manifestations of the post-polio syndrome

Author(s): Jubelt B, Cashman NR
Affiliation(s): Les Turner Amyotrophic Lateral Sclerosis Research Laboratory, Northwestern University Medical School, Chicago, Illinois

Publication(s): Critical Reviews in Neurobiology
Citation: Crit Rev Neurobiol. 1987;3(3):199-220

Patients with late effects of poliomyelitis, ie PPS, are being seen at an ever increasing frequency by general physicians, neurologists, and orthopedists. An appropriate time interval for the onset of late manifestations has elapsed since the major epidemics of poliomyelitis in the 1940s and 1950s. Post-polio neurological manifestations primarily include new weakness, atrophy, muscle pain, and fasciculations. Fortunately, the weakness is of a very slow, progressive nature. Abnormal laboratory studies include routine EMG, demonstrating chronic denervation; SFEMG, demonstrating increased fiber density, increased jitter, and blocking; and muscle biopsy most often revealing fiber-type grouping of chronic denervation and small isolated angular (or angulated) fibers and group atrophy in some series, both suggestive of active denervation. Unfortunately, both EMG and muscle biopsy studies suffer from a lack of specificity as they do not appear to distinguish asymptomatic from symptomatic (new weakness, PPMA) patients with prior poliomyelitis. Although the cause of PPMA is unknown, electrophysiological (SFEMG) and muscle biopsy studies suggest that the process involves a loss or dropout of axon terminals of reinnervated motor units. The axons terminal dropout could be due to dysfunction in the cell soma, the axon, or the terminals themselves. Whether motor neuron exhaustion, a persistent viral infection, or immune-mediated mechanisms play a role in the pathogenesis of the late weakness is unclear at present and will require further investigation. Treatment at this time is of a supportive nature. A major controversy involves the role of strengthening exercises in these patients since experimental animal studies suggest that excessive exercise of denervated muscles leads to increased weakness. Clearly, a better understanding of PPS and PPMA will allow more effective management of these patients’ problems and might also provide insight into other motor neuron and neuromuscular junction diseases.

Post-polio sequelae: physiological and psychological overview

Author(s): Frick NM, Bruno RL
Affiliation(s): Not stated

Publication(s): Rehabilitation Literature
Citation: Rehabil Lit. 1986 May-Jun; 47(5-6):106-11

When the Salk and Sabin vaccines brought an end to the annual summer nightmare of polio epidemics, most Americans simply forgot about polio. Even many of those who had paralytic poliomyelitis put the disease out of their minds once they had achieved maximum recovery of function. Unfortunately, polio has again forced itself into the nation's consciousness. Over the past five years, many of those who had polio have been experiencing new and unexpected symptoms that range in severity from being merely unpleasant to severely debilitating.

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A long-term follow-up study of patients with post-poliomyelitis neuromuscular symptoms

Author(s): Dalakas MC, Elder G, Hallett M, Ravits J, Baker M, Papadopoulos N, Albrecht P, Sever J
Affiliation(s): Not stated

Publication(s): The New England Journal of Medicine
Citation: N Engl J Med. 1986 Apr 10; 314(15):959-63

A “post-polio” syndrome characterized by new neuromuscular symptoms, including muscle weakness, may develop years after recovery from acute paralytic poliomyelitis. We studied 27 patients (mean age, 50.6 years) in whom new muscle weakness developed a mean of 28.8 years after recovery from acute polio. We reevaluated these patients during a mean follow-up period of 8.2 years (range, 4.5 to 20) after they were originally studied at the National Institutes of Health. The total mean follow-up period after the onset of new weakness was 12.2 years (range, 6 to 29). The patients were assessed with quantitative muscle testing, muscle biopsy, electromyography, and virologic and immunologic examination of the cerebrospinal fluid. Muscle strength had declined in all patients. The rate of decline averaged 1 percent per year. The decrease was irregular, with subjective plateau periods that ranged from 1 to 10 years. None of the patients had amyotrophic lateral sclerosis. Oligoclonal bands (IgG) were found in the cerebrospinal fluid of 7 of 13 patients studied, but no specific elevation of antibodies to poliovirus was observed in the cerebrospinal fluid. The newly affected muscles that were evaluated longitudinally with follow-up muscle biopsies and electromyography showed signs of chronic and new denervation. Groups of atrophic muscle fibers (group atrophy) and "neurogenic jitter" were not present. New post-polio muscle weakness is not a life-threatening form of motor-neuron deterioration. It appears that this weakness is not due to a loss of whole motor neurons, as in amyotrophic lateral sclerosis, but that it is due to a dysfunction of the surviving motor neurons that causes a slow disintegration of the terminals of individual nerve axons.

New problems in old polio patients: results of a survey of 539 polio survivors

Author(s): Halstead LS, Rossi CD
Affiliation(s): Not stated

Publication(s): Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):845-50

Presented are the results of a questionnaire survey on new health problems in 539 polio survivors. The most common new problems were fatigue, weakness in previously affected and unaffected muscles, muscle pain, and joint pain. The median time from polio to the onset of these problems ranged from 30 to 40 years. Factors at onset of polio most strongly associated with developing these new health problems were: being hospitalized, being over 10 years old, being on a ventilator, and having paralytic involvement of all four limbs. The differential diagnoses of these new problems, implications for treatment and areas for future research are discussed.

Post-polio sequelae and the psychology of second disability

Author(s): Frick NM
Affiliation(s): Not stated

Publication(s): Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):851-3

Thousands of persons who had poliomyelitis are reporting new physical symptoms that are eroding physical abilities, regained only after strenuous and lengthy rehabilitation, that were thought to have been permanently restored. These symptoms are causing persons to feel they are becoming disabled for a second time by the same disease. These new symptoms are psychologically traumatic also because they are unexpected, their cause is unknown and there is a lack of knowledge and understanding concerning them within the medical community. Society's negative and pejorative attitude toward the disabled is discussed to explain the psychological trauma associated with any first or second disability. Psychological processes that promote acceptance of disability are outlined with special emphasis on the post-polio experience. Post-polio support groups are described as one means to obtain the resources necessary to surmount the physical and psychological difficulties associated with post-polio sequelae.

Post-polio sequelae - differential diagnosis and management

Author(s): Maynard FM
Affiliation(s): Not stated

Publication(s): Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):857-61

Forty-two patients with a past history of poliomyelitis were evaluated at a post-polio clinic for new problems or impairments. Evaluation included a complete history, neurological and biomechanical examination and electrodiagnostic studies. Based on this evaluation patients were placed into three groups: 23 patients were considered to have or likely to have Progressive Post-Polio Muscular Atrophy (PPPMA); 17 patients were considered to have other post-polio sequelae; and two patients had problems unrelated to a past history of polio but mistaken for post-polio sequelae. Musculoskeletal pain was a common complaint among all groups of patients. Twenty-two of the 40 patients with post-polio sequelae were advised to alter their method of ambulation and/or decrease their activity pattern in order to decrease strain and/or excessive exertion of involved muscles. The role of chronic overuse and exercise in producing PPPMA or musculoskeletal pain problems is discussed. Characteristic clinical problems and useful management plans are described.

Vasomotor abnormalities as post-polio sequelae: functional and clinical implications

Author(s): Bruno RL, Johnson JC, Berman WS
Affiliation(s): Not stated

Publication(s): Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):865-9

Persons who had poliomyelitis report cold and discolored extremities and decreased muscle strength when exposed to mildly cool ambient temperatures. Bilateral digital cutaneous blood flow, skin temperature and median nerve latencies and amplitudes were measured at 30 degrees C, 25 degrees C and 20 degrees C in five post-polio subjects and age-matched controls. Cutaneous blood flow was lowest on the more affected side in the post-polio subjects but vasoconstriction with decreasing Ta was equal bilaterally in both groups. With decreasing Ta, median motor nerve latencies became clinically abnormal and “giant” sensory nerve potential amplitudes were seen in a majority of the post-polio subjects. It is concluded that an impairment of sympathetic vasoconstrictor outflow in post-polios allows passive dilatation and engorgement of the cutaneous venous capacitance beds. This promotes uncontrolled heat loss and causes cooling of nerve and muscle that is responsible for the impairment of muscle functioning and the abnormal electrophysiological findings.

Acute and latent effect of poliomyelitis on the motor unit as revealed by electromyography

Author(s): Wiechers DO
Affiliation(s): Not stated

Publication(s): Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):870-2

When polio virus attacks the motor neuron it may be completely destroyed, damaged, or unaffected. Muscle fibers of a destroyed motor neuron are orphaned or reinnervated. Nearby functioning motor units will then send terminal axon sprouts to reinnervate the orphaned muscle fibers. If there are too many orphaned muscle fibers and not enough surviving motor units to reinnervate them, the orphaned muscle fibers will continue to fibrillate until they atrophy and die. The resultant effect of poliomyelitis upon the affected muscle is an overall loss of motor units with the remaining units innervating many more muscle fibers than they originally did. There appears to be a late effect of polio upon these larger reinnervated motor units. After approximately 20 to 30 years, impulse transmission to the muscle fibers of the large reinnervated motor unit begins to fail. These transmission difficulties increase with age and time from recovery. These late onset transmission abnormalities may be factors in patient complaints of fatigue and progressive weakness.

Polio: long-term problems

Author(s): Perry J, Fleming C
Affiliation(s): Not stated

Publication(s): Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):877-81

The most common long-term problems seen in polio are brace problems, knee recurvatum, increasing weakness due to overuse and ankle equinus. A definite increased incidence of problems is seen after the patient is more than 30 years post-polio. The basis for most of these problems is chronic mechanical strain of weak musculature and substituting ligaments. Overuse can cause increasing weakness resulting in pain and decreasing function. It is therefore important to follow polio patients closely, especially those that are more than 30 years post-polio. If signs of overuse or chronic mechanical strain are noted, treatment should not be delayed.

Polio residuals clinic: conditioning exercise program

Author(s): Owen RR, Jones D
Affiliation(s): Not stated

Publication(s): Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):882-3

The additional disability experienced by individuals who had poliomyelitis many years earlier has a variety of expressions and a variety of interacting origins. Undertraining and deconditioning are addressed in this article. Weakened musculature often fatigues before a conditioning level of activity is reached. An adapted exercise program for cardiac endurance will reduce symptoms of fatigue and pain. An intentional training program for muscles weakened further by disuse or underutilization will supplement the conditioning program. The clinical assessment and exercise prescription is described.

The use of strengthening exercises in post-polio sequelae - methods and results

Author(s): Feldman RM
Affiliation(s): Not stated

Publication(s): Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):889-90

Some individuals who had poliomyelitis 20 to 30 years ago are now reporting a recurrence of symptoms of weakness in the same muscle groups that were weakened during the initial onset of the disease. Electrophysiological findings on EMG and repetitive stimulation studies identify changes peculiar to this disease. Non-fatiguing progressive resistive exercises have been used to strengthen muscle groups demonstrating this secondary weakness after the muscles have been identified by electrophysiological studies. Favorable results are reported after non-fatiguing exercises which, combined with Occupational Therapy and appropriate orthotic management, have resulted in improvement in function of ambulation and activities of daily living. The causes of muscle atrophy and pain seen in these individuals are also discussed.

Poliomyelitis: late respiratory complications and management

Author(s): Fischer DA
Affiliation(s): Not stated

Publication(s): Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):891-4

One hundred forty-six respiratory polios have been reviewed for complications and current respiratory aids. One hundred thirty-eight of these people required respirator assistance at the onset of their poliomyelitis infection. Fifteen patients have kyphoscoliosis. Seventy-five percent of the total patients require some assisted ventilation. Fifty-two percent have tracheostomies. Most of these people have been followed at Rancho Los Amigos Medical Center for many years. The literature regarding late respiratory complications of polio is reviewed.

Late postpoliomyelitis muscular atrophy: clinical, virologic, and immunologic studies

Author(s): Dalakas MC, Sever JL, Madden DL, Papadopoulos NM, Shekarchi IC, Albrecht P, Krezlewicz A
Affiliation(s): Not stated

Publication(s): Reviews of Infectious Diseases
Citation: Rev Infect Dis. 1984 May-Jun; 6 Suppl 2:S562-7

Seventeen relatively young patients, ages 31-65 years (average, 45) with prior poliomyelitis, who after a number of years of stability had experienced new neuromuscular symptoms, were studied. Seven patients had deterioration of functional capacity and then stabilization without new muscular weakness. The other 10 had late postpoliomyelitis muscular atrophy (late PPMA) characterized by focal progressive muscle weakness, wasting, fasciculations, and muscle pains affecting previously spared muscles or muscles previously affected but recovered. Four patients with late PPMA had lymphorrhages or lymphocytic infiltrates in their biopsied muscle; three of three patients had oligoclonal IgG bands in their spinal fluid, and five had variable peripheral T lymphocyte-subset ratios. In one patient with late PPMA, antibodies to poliovirus were specifically elevated in the cerebrospinal fluid. Our findings indicate that new motor-neuron disease can occur in patients with prior poliomyelitis and that immunopathologic mechanisms may play a role.

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