Management of postpolio syndrome

Management of postpolio syndrome
Henrik Gonzalez, Tomas Olsson, Kristian Borg Lancet Neurol 2010; 9: 634–42
Postpolio syndrome is characterised by the exacerbation of existing or new health problems, most often muscle weakness
See Refl ection and Reaction
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 fi ndings, with exclusion
Division of Rehabilitation
of other disorders as causes of the new symptoms. The muscle-related eff ects of postpolio syndrome are possibly
Medicine, Department of
associated with an ongoing process of denervation and reinnervation, reaching a point at which denervation is no
Clinical Sciences, Danderyd
longer compensated for by reinnervation. The cause of this denervation is unknown, but an infl ammatory process is
Hospital (H Gonzalez MD,
K Borg MD) and Department of
possible. Rehabilitation in patients with postpolio syndrome should take a multiprofessional and multidisciplinary
Clinical Neurosciences, Centre
approach, with an emphasis on physiotherapy, including enhanced or individually modifi ed physical activity, and muscle
for Molecular Medicine
training. Patients with postpolio syndrome should be advised to avoid both inactivity and overuse of weak muscles.
(T Olsson MD), Karolinska
Institute, Stockholm, Sweden
Evaluation of the need for orthoses and assistive devices is often required.
Introduction
summary of the pathophysiology and clinical 12–20 million people worldwide have sequelae of characteristics of postpolio syndrome, outline diagnostic poliomyelitis, according to Post-Polio Health and treatment options, and suggest future research International. Postpolio syndrome is the most common strategies. neuromuscular disorder in Sweden1 and the most henrik.gonzales@telia.com
prevalent motor neuron disease in the USA.2 Pathophysiology
A common misconception is that people in developed Studies of motor units of patients with postpolio For more on Post-Polio Health
countries who have had poliomyelitis are old. Large syndrome have revealed an ongoing denervation– International see http://www.
epidemics were frequent in Europe and in the USA in reinnervation process.8,9 This is probably initiated after the 1940s and during the fi rst half of the 1950s when the acute poliomyelitis, and over time leads to increased vaccination programmes were launched,2,3 and many motor unit areas caused by collateral sprouting of people who have recovered from poliomyelitis infection adjacent motor neurons in the spinal cord in patients are still of working age. A global eradication of with postpolio syndrome; a process also evident during poliomyelitis is close to accomplishment; in a few years normal ageing, although not until the seventh decade of time there should be no new cases of the disorder. life.10 The motor unit area might increase by up to However, in several developing countries, many young 20 times, reaching a level at which further reinnervation people have recently contracted the disease. Although the is no longer possible. Uncompensated denervation number of people with sequelae of poliomyelitis will causes atrophy of muscle fi bres and subsequently loss of decrease in Europe and the USA in the next few decades, muscle strength.8,9 The underlying cause of the ongoing many people in developing countries will be aff ected for denervation resulting in the motor symptoms of at least a generation. postpolio syndrome is unclear. Several hypotheses have Many health-care workers believe that the sequelae of been proposed, as described below.
poliomyelitis do not change throughout a patient’s
lifetime. However, more than a century ago the Stress or overuse of motor units
development of new or exacerbated symptoms was Stress is a favoured hypothesis, proposed by Wiechers
already reported long after the acute poliomyelitis and Hubbell,11 which attributes postpolio syndrome to
infection.4 The existence of postpolio syndrome has been
degenerating terminal axonal sprouts and an inability to questioned, but the late eff ect of poliomyelitis, or maintain the increased metabolic demand from the postpolio syndrome, is generally accepted as a defi ned enlarged motor unit. Overuse was suggested by Perry clinical entity.2,5,6 The prevalence of postpolio syndrome and colleagues12 in 1988. Overuse of remaining motor has been reported to be between 20% and 85% of people units leads to activation of compensatory mechanisms: who have had poliomyelitis.2,6,7 This disparity is most muscle fi bre transition from fast to slow contracting
probably caused by the use of diff erent clinical diagnostic
muscle fi bres and muscle fi bre hypertrophy, that is, criteria. In this context, it is important to remember that changes in contractile properties of the overused muscle people who have sequelae of poliomyelitis but who do fi bres and an increase of the contractile tissue.8,13–15 not fulfi l diagnostic criteria for postpolio syndrome might Compensatory changes in the muscle have negative still have substantial loss of motor function and be in eff ects, such as muscle pain and fatigue caused by lower need of therapeutic interventions.
capillarisation and changes in the contractile properties Interest in postpolio syndrome has increased over the of the remaining muscle fi bres. These changes favour past two to three decades, with research varying in focus muscle strength before endurance,15 and thus a from molecular to clinical aspects, and health-related discrepancy between low energy resynthesis and a high quality of life. In this Review, we provide a comprehensive energy utilisation in the overused muscle fi bres, which is www.thelancet.com/neurology Vol 9 June 2010
of importance for development of fatigue, was assumed. Nonetheless, results from longitudinal studies do not Panel: Criteria for postpolio syndrome16
show that overuse leads to increased persistent muscle 1 Prior paralytic poliomyelitis with evidence of motor neuron loss, as confi rmed by history of the acute paralytic illness, signs of residual weakness and atrophy of muscles on neurological examination, and signs of denervation on The length of time between acute poliomyelitis and the start of symptoms is a risk factor for postpolio syndrome,17 2 A period of partial or complete functional recovery after and two studies independently concluded that age was a acute poliomyelitis, followed by an interval (usually confounding factor for the development of postpolio 15 years or more) of stable neurological function. syndrome.17,18 However, the normal ageing process can 3 Gradual or sudden onset of progressive and persistent also cause muscle weakness in patients with postpolio new muscle weakness or abnormal fatigability (decreased endurance), with or without generalised fatigue, muscle atrophy, or muscle and joint pain. (Sudden onset may Persistent virus
follow a period of inactivity, or trauma or surgery.) Less Persistence of poliovirus fragments might cause postpolio commonly, symptoms attributed to postpolio syndrome syndrome. Mutated poliovirus genomic sequences have include new problems with breathing or swallowing. been detected in the CSF of some patients,19,20 but not in the CSF of others.21 One study22 reported detection of 5 Exclusion of other neurological, medical, and orthopaedic poliovirus genome fragments in all patients with postpolio syndrome. Further study is clearly needed to prove the hypothesis of a persistent viral infection in postpolio syndrome. If the neurodegeneration in postpolio syndrome is an ongoing process caused by chronic infl ammation, Immunological factors and chronic infl ammation
treatment of the disorder with drugs that modify the Increasing interest in recent years has been devoted to immune response (eg, intravenous immunoglobulin and
the immunological process underlying postpolio cortisone, as discussed later) should be possible.
syndrome. Infl ammatory changes have been described
in the spinal cord23,24 and the CSF25 of patients with Genetics
postpolio syndrome. Also, concentrations of several Some studies have attempted to fi nd a genetic background
cytokines, mainly those with proinfl ammatory actions to postpolio syndrome. Bartholdi and colleagues32 reported
(eg, interferon-γ and tumour necrosis factor [TNF]), are that the SMN gene deletion, a cause of adult spinal
high in the CSF of patients.26–28 These fi ndings probably muscular atrophy, was not present in patients with
refl
ammation in the spinal cord postpolio syndrome. Polymorphisms in the Fc-gamma parenchyma, with potential damage to motor neurons. receptor IIIA might be a risk factor for postpolio
Further support for this scenario was obtained by use of syndrome.33 Further studies of possible gene variants that
unbiased proteomics of the CSF. A small series of could predispose to both acute poliomyelitis and postpolio
proteins and their fragments were found at high syndrome would be of interest.
concentrations in the CSF only in patients with postpolio
syndrome; the peptides were all involved in apoptosis Diagnosis
and infl ammation.29 Furthermore, peptide cleavage Several diagnostic criteria for postpolio syndrome have
patterns were consistent with actions of TNF. A systemic
been proposed.8,34–36 Diff erent criteria from diff erent count- proinfl ammatory response in postpolio syndrome is also ries focus on muscle atrophy34 or muscle dysfunction,10 but likely. High TNF concentrations in blood were associated all are mainly based on those originally suggested by with increased muscle pain.30 The fi nding of high titres Halstead and Rossi.36 In 1991, Halstead added gradual or of poliovirus antibodies and high numbers of regulatory abrupt onset of new neurogenic muscular weakness as a T cells31 further supports the notion of an active criterion.37 In the criteria suggested by the 29th European immunological process. Neuromuscular Centre workshop on postpolio muscle The role of this chronic infl ammation in postpolio dysfunction, clinical and neurophysiological fi ndings, syndrome is unclear and further study is needed. MRI, or both, compatible with a lower motor neuron lesion Potential causes include a late aberrant immune response were included.8 At the March of Dimes international to the original infection, a persistent immune response, conference on postpolio syndrome in 2000, diagnostic or persistence of viral particles (boosting of the response criteria were recommended, including a criterion that by non-polio enteroviruses might also be involved); a late symptoms should persist for at least 1 year (panel).16 autoimmune complication of the original infection; and Diagnosis of postpolio syndrome is based on clinical an immune response secondary to ongoing neuro- symptoms and signs (tables 1 and 2). To diagnose a patient with postpolio syndrome not all symptoms and signs www.thelancet.com/neurology Vol 9 June 2010
In a large cohort of patients with a reported history of Symptoms
Treatment
poliomyelitis, 5% had normal EMG.42 Sandberg and Physical activity, muscle training, avoiding muscle Stålberg42 suggested that, despite normal EMG, there was overuse and disuse, and lifestyle changes a transient functional loss without degeneration of weakness in previously clinically unaff ected muscle groups anterior horn cells, making these cells more vulnerable Symptomatic medication, physiotherapy, bracing, weight reductionDecompression and pharmacological treatment for Symptoms and signs
Because poliomyelitis aff ects the anterior horn cells in Physical activity, muscle training, rest, avoiding the anterior part of the spinal cord (the fi rst part of the muscle overuse and disuse, lifestyle changes, endurance. Mental fatigue can also weight reduction, and, if required, medication for motor unit), postpolio syndrome is a lower motor neuron restless legs syndrome, depression, and sleep disorder. The symptoms in patients with postpolio syndrome are muscle weakness and atrophy, and fatigue Assisted ventilation and inspiratory muscle training and pain from muscles and joints (table 1).2,37 Other muscles and chest wall deformities, Invasive controlled mechanical ventilationobstructive apnoea, and central muscle twitching and cramps, respiratory distress, and Instruction on swallowing and voice therapy On clinical investigation, signs of a lower motor neuron Table 1: Symptoms and treatment of postpolio syndrome
disorder are apparent; the most typical is asymmetrical fl accid paresis. Sensory defi cits are not observed unless a secondary disorder is present, for example compression Clinical signs
Investigation
Weak or no muscle strength. Lower Clinical examination Health-related quality of life has been investigated in patients with postpolio syndrome in several studies. Diff erent questionnaires, mainly the SF-3643–46 and the Nottingham health profi le,47–51 have been used. But, although diff erent questionnaires were used, results were comparable, with low health-related quality of life Neurophysiology (nerve conduction velocity) in physical domains, pain, and vitality; however, results Investigation on oesophageal and laryngeal from mental domains are similar to those reported by control individuals. Patients with postpolio syndrome Pulmonary function Weak respiratory muscles, chest Pulmonary investigation, including complete can have a high degree of independence with regard to and sleep-disordered wall and spinal deformities personal activities of daily living but typically have culty with mobility.52–54 Overall morbidity is high in Table 2: Clinical signs of postpolio syndrome
patients with postpolio syndrome compared with that in healthy control individuals.55 Besides, comorbidity and need to be present. As stated previously, this is a diagnosis ageing can cause further health problems in these based on exclusion. The most common symptom of patients.44
postpolio syndrome is a triad of fatigue, deterioration in
muscle strength, and pain (panel). Serum creatine Muscle weakness and atrophy
phosphokinase concentrations are usually normal.2,3
In patients with postpolio syndrome new or increased Diagnosis is supported by electrophysiological exam- muscle weakness and atrophy can be permanent, because ination including electromyography (EMG), which usually of loss of motor units, or transient, because of muscle reveals longstanding neurogenic signs. Macro-EMG can fatigue.2 Progression of muscle weakness is probably slow enable a more precise quantifi cation of remaining motor but faster than in normal ageing.56 Stolwijk-Swüste and units and their size, thereby indicating the extent of the colleagues57 systematically analysed studies assessing the denervation and reinnervation process.38 course of functional status and muscle strength over time Patients who had non-paralytic polio and those with in patients with postpolio syndrome. Only two studies that clinically unaff ected muscles and a history of polio- myelitis might present with postpolio syndrome and they reported inconsistent results. Four studies that neuro physiological signs of denervation.39,40 In people assessed muscle strength were of suffi aged 60 years or over, loss of half of their motor units reported a decline in muscle strength and two reported no might not be associated with a decline in muscle change. Grimby and colleagues58 reported a decrease of strength;41 this could explain both why paresis might not muscle cross-sectional area in patients with poliomyelitis be recognised and the occurrence of new postpolio sequelae during a 4-year follow-up period58 and a 9–15% syndrome symptoms in extremities that were thought decrease in muscle strength during a follow-up period of not to have been aff ected by poliomyelitis.
8 years.9 We recorded a 6% decrease in muscle power in a www.thelancet.com/neurology Vol 9 June 2010
selected muscle in patients with postpolio myelitis over a age and time since the acute poliomyelitis) are non-6-month period.43 McComas and colleagues59 described a modifi able, others (eg, stress and physical activity) are decrease in motor units of 13·4% in patients with previous modifi able, and hence they are important to consider in poliomyelitis during a 2-year follow-up. Lower fi gures the management of these patients.
were reported by Sorenson and coworkers,60 with a yearly
loss of 3% during a 15-year follow-up period, and Ivanyi Pain
and colleagues61 reported no decrease of muscle strength Pain is common in patients with postpolio syndrome; the
during a follow-up period of about 2 years. Thus, as pain intensity is high76 and is more often located in parts
pointed out by Stolwijk-Swüste and colleagues,57 long-term
of the body that were aff ected by polio than in parts that follow-up studies with unselected study populations and were not.77 Many patients with postpolio syndrome report age-matched control individuals are needed to shed cramping pain in the legs (most often the upper leg further light on this question.
musculature) and aching pain in the neck and shoulders.77 Loss of motor units and a decrease in muscle strength The pain has been reported to be widespread.78 Pain is and endurance is not necessarily associated with physical most common in women with postpolio syndrome, young function, physical activity, or social participation. There is patients, or patients who have had a long stable period.79 no correlation between muscle strength and walking Although the pain can have variable origin, it is mostly ability in patients with postpolio syndrome.50,62 The associated with overload of muscles, tendons, and joints association is non-linear possibly because of compensatory and is related to the amount of physical activity.77,79–82 10% of patients with postpolio syndrome have neuropathic Daily activity did not decrease during a 3-year follow-up pain, caused by secondary disorders such as nerve period63 and participation in walking activities was self- compression or disc hernia that can be treated.83 Thorough cient for daily life mobility by the clinical assessment of patients with postpolio syndrome patients.64 Horemans and colleagues50 and Klein and who have neuropathic pain is therefore needed to exclude
colleagues63 found that actual walking in daily life in concomitant and secondary disorders and to provide
patients with postpolio syndrome was not only aff ected adequate pain treatment.
by walking capacity but also by social behaviour and
personal lifestyle. Willen and colleagues65 reported that Hypoventilation
there was a non-linear relation between walking speed Some patients with polio who had initial weakness of
and muscle strength. Furthermore, Sorenson and respiratory muscles or other factors that might have
colleagues66 concluded that there was no association decreased lung function (eg, chest deformity or
between the amount of decline in motor units and the progression of scoliosis) experience new respiratory
symptoms of this late muscle deterioration, but minor problems as a consequence of postpolio syndrome.84
changes in disability were seen during follow-up.
Furthermore, patients with postpolio syndrome are also Increased muscle weakness can lead to reduced balance at risk of nocturnal hypoventilation caused by sleep- and increased numbers of falls in patients with previous disordered breathing (a general term used for diff erent poliomyelitis.67 Patients with increased muscle weakness kinds of symptoms related to disorders of breathing might need to use assistive devices for walking or a during sleep, such as obstructive apnoea, central apnoea, wheelchair, and those with weak respiratory muscles or a mixed dyspnoea). Shortness of breath is the most might require ventilation. common complaint, but patients might also present with non-specifi c symptoms, such as daytime somnolence, morning headache, and fatigue.84–87 In patients with a Fatigue in patients with postpolio syndrome is multi- history of these symptoms, hypoventilation and sleep- dimensional68 and can be of general or mental character disordered breathing should always be investigated with
derived from the CNS (caused by early neuronal damage tests of pulmonary function and polysomnography,
in the brain in the acute poliomyelitis stage, overlapping respectively. Cardio pulmonary comorbidity should also
psychological factors, or both) or muscular from the be considered.
motor unit.69–71 Fatigue is probably the most disabling
symptom of postpolio syndrome. Muscle weakness Other symptoms
during fatigue is caused by slow recovery of the muscle Other symptoms and new health problems include cold
and could refl ect both central and peripheral fatigue.2,72,73
intolerance, cold sensitivity, muscular twitching, and Fatigue in postpolio syndrome has a negative eff ect on muscular cramps, as well as concentration problems and physical and psychosocial functioning but does not swelling of legs and feet.7,17,88,89 Bulbar muscle weakness impair mental health.74,75 Health-related quality of life for can lead to dysphagia and dysphonia.2,3,7,90,91 Investigation vitality is to a greater extent caused by physical of whether new health problems are caused by postpolio (eg, decreased physical endurance) than by mental syndrome, are secondary to postpolio syndrome, or are (eg, mental fatigue) parameters.75 According to Trojan and caused by comorbidities is important because some colleagues,68 although some risk factors for fatigue (eg, comorbidities require adequate intervention.
www.thelancet.com/neurology Vol 9 June 2010
Clinical management
today. Heavy resistance training was recommended for No specifi c treatment for postpolio syndrome exists. patients with postpolio syndrome who had near-normal Evaluation of the effi cacy of treatment regimens for muscle strength and no signs of reinnervation of the patients with postpolio syndrome was initiated by motor unit.8,101 Submaximum endurance training, which the Cochrane Library in 2009. Patients with postpolio increases muscle strength and endurance,98 was syndrome often have a wide range of medical recommended for patients with moderate paresis and problems and may also have associated problems with signs of reinnervation of the motor unit.8,101 Patients with participation in society, such as need for technical aids severe paresis should avoid muscle training; however, at home and at work.
fi nding a suitable physical activity for cardiovascular conditioning in these patients is important. Aerobic Physiotherapy, physical activity, and muscle training
exercise, including bicycle ergometry, treadmill walking, Physical activity forms the basis of management of patients and swimming, are recommended.2 Aquatic exercise is with postpolio syndrome. Most patients with postpolio also benefi cial.102
syndrome benefi t from appropriate physical activity and a
large proportion benefi t specifi cally from individually Pharmacological treatment
chosen muscle training. Lygren and colleagues78 noted that
A few controlled trials of pharmacological treatments patients with postpolio syndrome who reported doing have been done in patients with postpolio syndrome. regular physical activity had fewer symptoms and a higher Amantadine and high-dose prednisone were ineff ective at level of functioning than those who were not often reducing muscle weakness and fatigue.103,104 In one study, physically active. No prospective data show that increased no benefi cial eff ect on muscle function was reported after physical activity leads to muscle weakness.6 treatment with pyridostigmine.105 Conversely, another Few randomised controlled trials have investigated the study reported a slight improvement in walking in eff ect of muscle training in patients with postpolio patients treated with pyridostigmine.106 In another syndrome. Because most patients with postpolio randomised controlled trial, there was no signifi cant syndrome have asymmetrical muscle weakness and the diff erence in muscle function between patients with degree of paresis diff ers over time and between patients, postpolio syndrome who were having muscle training training programmes should be carefully customised and and taking coenzyme Q-10 and those receiving placebo.107 planned by physiotherapists to avoid both overuse and Modafi nil has proven ineff ective for treatment of fatigue disuse, and the level of physical activity modifi ed to in patients with postpolio syndrome in two randomised decrease pain.77,92 Patients should be carefully monitored controlled trials.108,109 However, a controlled study on the to identify signs of increasing muscle weakness and eff ect of lamotrigine on pain, fatigue, and quality of life muscle pain, and thus to avoid adverse eff ects, especially had promising results.110overuse of muscles that are clinically unaff ected but were Based on the notion of an infl ammatory process in the found clinically or by use of EMG to be aff ected by polio CNS of patients with postpolio syndrome, intravenous infection.12,16 Overuse-induced weakness has been immunoglobulin has been tested. A single course of proposed,12–15 but there is no evidence that overuse high-dose intravenous immunoglobulin greatly reduced weakness permanently damages muscles aff ected by the concentrations of proinfl ammatory cytokines in the poliomyelitis. Rather, the occurrence of overuse-induced CSF 2  months after treatment.27 The idea of a role for weakness could be explained by metabolic demands these cytokines in postpolio syndrome would be exceeding the individual muscle capacity or reparable strengthened if a clinical response was also seen, with intramuscular damage, similar to those in healthy amelioration of symptoms and signs. In a single patient,111 individuals.93 In this respect, one must also bear in mind in small open studies,27,112 and in two larger randomised that the energy cost of movement is higher in patients controlled trials,26,43 variable eff with postpolio syndrome than in control individuals.94,95 immunoglobulin on muscle strength, pain, physical To save energy, reduction in activity and pacing as well activity, and quality of life have been described. These as training in muscle conservation techniques are data encourage further controlled trials to more fi rmly required; this approach remains the mainstay of treatment establish if this treatment regimen should be used. of postpolio syndrome.16,96 Cup and colleagues97 reviewed Furthermore, such trials should assess dosing and dosing studies dealing with exercise therapy in patients with intervals and ways to predefi ne responders and non-postpolio syndrome and found evidence for eff ectiveness responders to this treatment.
of strengthening or aerobic exercise to be insuffi Symptomatic treatment is of course important in However, endurance98,99 and heavy resistance training100 postpolio syndrome. For example, restless legs syndrome are eff ective at increasing muscle strength and endurance is a common complaint in patients with postpolio in patients with postpolio syndrome. In 1994, the syndrome and can be successfully treated with dopamine European Neuromuscular Centre workshop8 on postpolio agonists.113 As pointed out by Trojan and colleagues,68 muscle dysfunction and Grimby and Stålberg101 the most prominent symptom in postpolio syndrome, recommended guidelines for exercise that are still valid fatigue, is multidimensional, and some of the con- www.thelancet.com/neurology Vol 9 June 2010
tributing factors can be treated. Other symptomatic syndrome have a higher risk for late poliomyelitis-related pharmacological treatments that should be considered symptoms compared with active people with postpolio are analgesics and antidepressants.
syndrome.122 An active lifestyle should therefore be recommended. However, patients should be carefully assessed to avoid both overexertion and inactivity. Joint deformities, arthrosis, and limb-length inequality Lifestyle modifi cations can be eff ective in reducing may require surgery. Increased function can be achieved overuse symptoms.123 Workload and social interaction in by arthrodesis, tendon transfers, and muscle transplant- the community68 should be assessed, and occupational ation.114 For other secondary disorders, such as spinal and vocational interventions might be needed.124 Weight stenosis and disc hernia, surgical treatment can help. reduction or a programme to stabilise bodyweight may However, motor neurons of patients with postpolio be important.3,7 A weight reduction programme, when syndrome might be more sensitive to eff ects of indicated, can reduce fatigue, improve respiratory anaesthetic drugs and thus patients should be carefully symptoms, and increase mobility.
assessed before and monitored both during an operation Occupational interventions, weight-control programmes, group therapy (eg, water exercise125), and increased use of assistive devices should be considered and managed by Orthoses and assistive devices
the multiprofessional rehabilitation team.126 Orthopaedic braces can restrict unwanted movements—
supporting joints and muscles and reducing the impact Future perspectives
of bodyweight, particularly in legs and feet. The use of Better distinction between postpolio syndrome and
braces should improve mobility, reduce pain, and reduce
poliomyelitis-related secondary disorders by more overuse of the still well functioning parts of the muscles, thorough clinical analyses is important. Further studies joints, tendons, and ligaments. Use of lightweight knee– are needed to establish if there are clinical markers or risk ankle–foot braces can have a benefi cial eff ect (measured factors that might predict the development of postpolio as decreased oxygen consumption during walking) and syndrome. If identifi ed, measures for preventing post-save energy.116,117 polio syndrome could be started at an early stage. Further Patients with postpolio syndrome should be supplied study of subgroups of patients with postpolio syndrome with appropriate orthoses and braces on the basis of (eg, assessing rate of progression of muscle weakness individual needs.118 To further improve energy effi ciency, and diff erent forms of fatigue) and identifi cation of carbon-composite material is preferred.116,117,119 Besides measures to distinguish these subgroups early and thus orthoses, assistive devices to increase functional activity be able to tailor treatment accordingly are also important. include crutches, manual and electrical wheelchairs, and Pharmacological and rehabilitation interventions seem eff ective in patients with postpolio syndrome. The study Thorén-Jönsson54 noted that, despite the need for of combinations of these diff erent therapeutic strategies assistance with mobility, patients with postpolio syndrome will be important to achieve the best outcomes. associated the use of assistive devices with negative Postpolio syndrome is likely caused by ongoing feelings. However, technical developments, including neurodegeneration, perhaps driven by aberrant chronic new lightweight and strong materials, have made orthoses and assistive devices more appealing to patients and therefore more useful. Kelley and DiBello118 have Search strategy and selection criteria
developed a patient classifi cation system that is useful for References for this Review were identifi ed through searches of The Cochrane Library (1950 to April, 2010), Medline (1950 to April, 2010), Embase (1974 to May, 2009), the Allied and Hypoventilation and ventilator support
Complementary Medicine Database (1985 to April, 2010), Some patients with hypoventilation need ventilator CINAHL (1984 to April, 2010), and Web of Science (1945 to assistance. Non-invasive positive pressure ventilation or April, 2010) with the search terms “post-poliomyelitis non-invasive bi-level positive airway pressure ventilation syndrome”, “poliomyelitis survivor”, “late onset poliomyelitis”, improve quality of life and are preferred by patients, “late eff ect poliomyelitis”, “post-poliomyelitis”, rather than invasive ventilators.120 Inspiratory muscle “complications after poliomyelitis”, and “disabilities after training can increase respiratory muscle endurance and poliomyelitis”. The searches were broad and all the terms were wellbeing; however, those patients with chronic truncated and diff erent proximity operators were used. No respiratory failure are still treated with controlled language restriction was made. Papers were also identifi ed mechanical ventilation through tracheostomy.121 through searches of the authors’ own fi les. We mostly selected publications from the past 6 years, but we did not exclude Lifestyle changes
important older publications. The reference list was modifi ed Lifestyle changes are necessary for many patients with according to recommendations from peer reviewers.
postpolio syndrome. Inactive patients with postpolio www.thelancet.com/neurology Vol 9 June 2010
infl ammation. More eff ective methods to halt the 15 Larsson L, Li X, Tollback A, Grimby L. Contractile properties in single muscle fi bres from chronically overused motor units in relation to motoneuron fi ring properties in prior polio patients. syndrome will probably require a deeper understanding J Neurol Sci 1995; 132: 182–92.
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Confl icts of interest
23 Kaminski HJ, Tresser N, Hogan RE, Martin E. Spinal cord HG has received fees as a senior consultant for Pharmalink. TO has histopathology in long-term survivors of poliomyelitis. Muscle Nerve received fees for consultancy from Sanofi -Aventis, Merck Serono, and 1995; 18: 1208–09.
Biogen Idec. KB has received consultancy fees from Pharmalink. 24 Pezeshkpour GH, Dalakas MC. Pathology of spinal cord in Acknowledgments
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