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Europeanpolio.eu

Chapter 18
Post-polio syndrome
E. Farbu,1 N. E. Gilhus,2 M. P. Barnes,3 K. Borg,4 M. de Visser,6 R. Howard,7 F. Nollet,6
J. Opara,8 E. Stalberg9
1Stavanger University Hospital, Norway; 2University of Bergen, and Haukeland University Hospital, Bergen, Norway;
3Hunters Moor Hospital, Newcastle upon Tyne, UK; 4Karolinska Intitutet/Karolinska Hospital, Stochkholm, Sweden;
6University of Amsterdam, 1100 DD Amsterdam, The Netherlands; 7St Thomas' Hospital, London, UK; 8Repty Rehab Centre.
ul. Sniadeckio 1, PL 42-604 Tarnowskie Góry, Poland; 9University Hospital, S-75185 Uppsala, Sweden
1 Prior paralytic poliomyelitis with evidence of motor
neuron loss, as confirmed by history of the acute para-
The aim was to revise the existing EFNS task force docu- lytic illness, signs of residual weakness, and atrophy of ment, with regard to a common definition of PPS, and muscles on neurological examination, and signs of evaluation of the existing evidence for the effectiveness denervation on electromyography (EMG).
and safety of therapeutic interventions. By this revision, 2 A period of partial or complete functional recovery
clinical guidelines for management of PPS are provided.
after acute paralytic poliomyelitis, followed by an interval (usually 15 years of more) of stable neurologic function.
3 Gradual or sudden onset of progressive and persis-
tent muscle weakness or abnormal muscle fatigability
Many previous polio patients experience new muscle (decreased endurance), with or without generalized weakness, fatigue, myalgia and joint pain, and cold intol- fatigue, muscle atrophy, or muscle and joint pain. erance, and develop new atrophy several years after acute (Sudden onset may follow a period of inactivity, or paralytic poliomyelitis. The first case of new atrophy and trauma, or surgery.) Less commonly, symptoms attrib- weakness many years after acute paralytic polio was first uted to PPS include new problems with swallowing or described in 1875 by Raymond [1, 2].
The term post-polio syndrome (PPS) was introduced 4 Symptoms persist for at least 1 year.
by Halstead in 1985 [3]. In general, PPS has been inter- 5 Exclusion of other neurologic, medical, and orthopae-
preted as a condition with new muscle weakness or fati- dic problems as causes of symptoms.
gability in persons with a confirmed history of acute The symptoms reported for PPS are the same from all paralytic polio, usually occurring several decades after parts of the world. Muscle weakness, atrophy, generalized the acute illness.
fatigue, post-exercise fatigue, muscle pain, fasciculations, As PPS is reckoned to be a chronic disease, the EFNS cramps, cold intolerance, and joint pain dominate[3, task force on post-polio syndrome recommends that the 5–12]. A history of previous paralytic polio seems to criteria published by March of Dimes (MoD) in 2000 [4] increase long-term mortality [13].
should be regarded as universal criteria for PPS.
Deterioration of the neuromuscular function, overuse of motor units, the general ageing process, and inflam-matory changes in the central nervous system and serum, European Handbook of Neurological Management: Volume 1. have all been proposed as possible explanations for the Edited by N. E. Gilhus, M. Brainin and M. P. Barnes. new symptoms [14–17]. However, the underlying mech- 2011 Blackwell Publishing Ltd.
anisms are not fully elucidated, and we cannot conclude Gilhus—European Handbook of Neurological Management: Volume 1.
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which underlying factors are causing PPS. Regarding the role of clinical neurophysiology
clinical course, there is increasing evidence that the decline in muscle strength is slow with modest effect on Clinical neurophysiology is used for four main reasons. functional tasks [18, 19]. Generalized fatigue is a common First, to establish typical lower motor neuron involve- complaint among PPS patients, and not well understood, ment (neurogenic EMG findings, normal findings of although both neuromuscular decline and increased the sensory and motor nerves except for parameters levels of inflammatory cytokines indicate that fatigue in reflecting muscle atrophy). Second, to exclude other PPS should be reckoned as a physical entity [16, 20]. causes. This is part of the PPS definition, and it is From a clinical point of view it should be emphasized not uncommon to find patients in whom the initial diag- that comorbidity could cause PPS- mimicking condi- nosis of polio must be revised. Third, to find concomi- tions, requiring other investigations and treatments tant nerve or muscle disorders, such as entrapments and [10, 21–23].
radiculopathies. Fourth, to assess the degree of motor The prevalence of PPS has been reported from 15 to neuron loss. This cannot be quantified clinically, since 80% of all patients with previous polio, depending on the loss of neurones may be completely masked by compen- criteria applied and population studied [5, 10, 24–28]. In satory nerve sprouting and muscle fibre hypertrophy. many population-based studies, terms such as ‘late-onset Macro EMG studies have shown that loss of up to 50% polio symptoms' have been used instead of PPS. Hospital- of neurons may be compatible with a normal clinical based studies use the term PPS, but in these studies it is picture [15].
always debatable whether the patient material is repre- In longitudinal studies with macro EMG, a continuous sentative. Exact prevalence of PPS is therefore difficult to loss of neurons is demonstrated with exaggerated speed establish. For European populations, one Dutch study compared to normal age-dependent degeneration[34]. reported a prevalence of late-onset polio symptoms of New weakness appears when the compensatory mecha- 46%, one study from Edinburgh reported a prevalence of nisms are no longer sufficient, and occurs when macro more than 60%, in Estonia a prevalence of 52% has been motor unit potential (MUP) exceeds 20 times the normal reported, Norway 60%, and Denmark 63% [5, 29–31].
With regard to symptomatic treatment and clinical purposes, the difference between stable muscle weakness after polio and PPS seems as yet rather irrelevant. Still, it would be of great benefit to have a consensus on the term PPS for research and when evidence-based therapeutic MEDLINE via Pubmed, EMBASE, ISI, and Cochrane interventions have become available. The MoD criteria were searched with time limits 1966 until 2004. Search are based on the principle of exclusion of other causes terms were post-polio syndrome, postpoliomyelitis, for new deterioration, where PPS is characterized with PPMA, PPMD, poliomyelitis, in combination with man- new muscle weakness or abnormal muscle fatigability, agement, therapy, treatment, medicaments, physiother- and persistence of symptoms for at least 1 year. The apy, and intervention.
diagnosis of PPS is an exclusion diagnosis with no test or In the present revised document, the database search analysis specific for PPS, and the role of the investigation was supplied with the years 2004–2009.
is to rule out every other possible cause for the new No meta-analyses of intervention for PPS were found symptoms and clinical deterioration.
when searching the databases, but one Cochrane review Many patients report a sense of weakening in the is being prepared [35]. Data were classified according to muscles before it is detectable by clinical examination, their scientific level of evidence as Class I–IV [36]. Rec- although dynamometric muscle strength evaluation and ommendations are given as Level A–C according to the computed tomography (CT) imaging may be helpful scheme for EFNS guidelines. When only Class IV evi- [32, 33]. Atrophy is the end stage of new neuromuscular dence was available but consensus could be reached deterioration and by using this as a necessary criterion, the task force gives our recommendations as Good Prac- patients in an earlier stage of neuromuscular deteriora- tice Points (GPP)[36]. Consensus was reached mainly tion will be excluded.
through email correspondence.
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A questionnaire about diagnosis, management, and Several reports of increased levels of inflammatory care of post-polio patients was answered by the group markers in serum and CSF in PPS have raised the ques- members from The Netherlands, Norway, Poland, tion whether immunological changes could be a part of Sweden, and the United Kingdom in the first version; this the pathophysiology in PPS [16, 17, 46]. These findings has not been repeated in this revision.
have also presented a rationale for investigating immune-modulating therapies in PPS. Intravenous immunoglob-ulin (IVIg) has been tried in three therapeutic intervention studies. Gonzalez et al. performed a multicentre double-blinded randomized study including 135 patients where the primary endpoints were muscle strength in a pre-selected muscle group and quality of life measured with Acetylcholinesterase inhibitors, steroids,
the SF-36 scale. The patients were treated with either amantadine, modafinil, lamotrigine, coenzyme
placebo or 90 g IvIg, repeated after 3 months. A signifi- Q10, intravenous immunoglobulin (IVIg)
cant difference on muscle strength was found, but no The effect of acetylcholinesterase inhibitors in PPS significant effect on the SF-36 on pain, balance, or sleep has been investigated in four studies with particular quality[47]. Farbu et al. performed a double-blinded ran- emphasis on fatigue, muscular strength, and quality of domized study with 20 patients with the primary end- life. One open pilot study indicated a positive effect on points muscle strength (isometric), pain (VAS), and fatigue [37, 38], but this was not confirmed in two dou- fatigue (FSS) after 3 months [48]. The patients were ble-blinded randomized controlled trials using a daily treated with one dose of IvIg (2 g/kg body weight). A dose of 180 mg pyridostigmine [39, 40]. Horemans et al. significant effect was found on pain, but not on muscle reported a significant improvement in walking perfor- strength or fatigue. One open study with 14 patients mance, but the difference in quadriceps strength was not explored the effect of 90 mg IvIg on muscle strength, significant as reported by Trojan et al. Hence, there is physical ability measured by walking test, and quality of evidence at Class I that pyridostigmine is not effective in life (SF-36) [49]. There was a positive effect on quality the management of fatigue and muscular strength in of life, but not on muscle strength or physical ability. PPS. One randomized study explored the effect on These studies indicate that IvIg could have a modest fatigue of 400 mg modafinil daily in PPS [41]. Modafinil therapeutic benefit in PPS, but they include a small was not superior to placebo, and there is Class I evidence number of patients, the results are diverging according that modafinil is not effective on fatigue in PPS. There to which symptoms improving after treatment, and the are two randomized placebo-controlled studies investi- IvIg has not been compared with other therapies like gating the effect of high-dose prednisolone (80 mg daily) specific training programmes. There is also a remaining and amantandine (200 mg daily) on muscular weakness question about the appropriate dose of IvIg and thera- and fatigue (prednisone), and fatigue (amantadine) [42, peutic interval. Hence, IvIg can at present not be recom- 43]. They included a small number of patients, 17 and 23 mended as standard treatment in PPS, despite two Class respectively, and only Stein et al. included statistical 1 studies [50].
power calculations. There was no significant effect on muscular strength or fatigue in any of these Class I studies.
Lamotrigine has been tried in an open study (15 It has been claimed that muscular overuse and training patients treated with 50–100 mg lamotrigine daily), may worsen the symptoms in patients with residual where a positive effect on quality of life (Nottingham weakness after paralytic polio, and even provoke a further Health Profile), pain (VAS), and fatigue (FSS) was found loss of muscular strength.[51]. Many post-polio patients after 2 and 4 weeks [44]. A double-blinded randomized have been advised to avoid muscular overuse and inten- study is needed to confirm this finding. In a randomized sive training [4, 52]. Studies of morphology and oxidative double-blinded pilot study of coenzyme Q10 (200 mg capacity in the tibialis anterior muscle indicate a high daily) including 14 patients, no additional effect to resis- muscular activity due to gait and weight bearing [53, 54]. tance muscle training was found [45].
When followed prospectively, the macro EMG motor Gilhus—European Handbook of Neurological Management: Volume 1.
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unit potential amplitude (MUP) in the tibialis anterior maximal work load, intermittent breaks, and rest periods muscle was found to be increased after 5 years, whereas between exercise sessions to prevent the likelihood of there was no change in the macro MUP amplitude in overuse effects. This is an important aspect for PPS the biceps brachii muscle [55]. This indicates a more patients in general. Most of the participating patients in pronounced denervation-reinnervation process in the these studies younger than 60 years, and the effect of tibialis muscle, which may be due to daily use and higher exercise programmes older patients is therefore less muscle activities in the leg muscles. However, there are no prospective studies, which show that increased One randomized controlled study of post-polio muscle activity or training lead to loss of muscular patients with pain, weakness, and fatigue in their shoul- strength compared to absence of training or less muscu- der muscles compared the effect of exercise only, exercise lar activity. On the contrary, patients who reported in combination with lifestyle modification, and lifestyle regular physical activity had fewer symptoms and a modification only [68]. Significant improvement was higher functional level than physically inactive patients found only for the two groups with exercise (Class II). [11, 56]. One randomized controlled trial reported The endpoints in this study were combinations of significant improvement in muscular strength after a 12- several symptoms. Further studies are needed to identify week training programme with isometric contraction improvement on particular symptoms before conclu- of hand muscles [57]. Non-randomized trials with train- sions are drawn regarding lifestyle modifications.
ing programmes lasting from 6 weeks to 6 months involving both isokinetic, isometric, and endurance mus- Treatment in a warm climate and training
cular training have shown a significant increase in both isokinetic and isometric muscle strength [58–61]. Oncu Anecdotal reports from post-polio patients indicate a et al. found that both time-limited hospital and home positive effect of a warm climate and of training in warm exercise programmes improved fatigue and quality of life water with respect to pain and fatigue. One randomized in the short term [62]. No complications or side effects controlled study reported a significant reduction in were reported. Hence, there is evidence at Class II and III pain, health-related problems, and depression for both that supervised training programmes increase muscle groups after completing identical training programmes strength and can improve quality of life and relieve in either Norway or Tenerife [69]. No significant differ- fatigue in patients with post-polio syndrome. Two fol- ence in walking tests was seen. Both groups improved low-up open studies of multidisciplinary rehabilitation their walking skills, reduced their level of fatigue, depres- report a positive effect on fatigue and physical capacity sion, and health-related problems. However, the effect up to 1 year after the intervention [63, 64]. This is prom- remained significantly longer in the Tenerife group ising, but long-term effects (several years) of training are not documented and deserve prospective studies. For Dynamic non-swimming water exercises for post- patients without cardiovascular disease, one randomized polio patients have been reported to reduce pain, improve controlled study reported improved cardiovascular cardiovascular conditioning, and increase subjective fitness after supervised exercise programmes using wellbeing in a controlled but not randomized study ergometer cycle [65] (Class I). Aerobic training in upper (Class III) [70]. A qualitative interview study (Class IV) extremities had a beneficial effect on oxygen consump- indicated a positive effect on the self-confidence when tion, minute ventilation, power, and exercise time [66] performing group training in water [71].
(Class II). Aerobic walking exercises can help to econo-mize movements and increase endurance without improvement in cardiovascular fitness [67]. Ernstoff et al. reported an increase in work performance by Reduced pulmonary function due to weak respiratory reduction of heart rate during exercises; hence endurance muscles and/or chest deformities may occur in patients training seems to improve cardiovascular conditioning with previous polio [22, 72]. Patients with chest deformi- (Class IV). It is important to emphasize that most exer- ties have an increased risk of nocturnal hypoventilation cise studies have been executed with supervision, sub- and sleep-disordered breathing [22, 73, 74]. The preva- Gilhus—European Handbook of Neurological Management: Volume 1.
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lence of respiratory impairment is highest among patients proper orthoses, walking sticks, and wheelchairs facilitate who were treated with artificial ventilation in the acute daily life activities (GPPs). Participating in muscle-train- phase [22]. Shortness of breath is a common complaint ing programmes and endurance training will, in many in many post-polio patients, but is not necessarily related cases, also lead to weight loss, but there is no evidence to respiratory impairment, but rather to orthopaedic and that weight reduction alone can ameliorate symptoms. general medication problems. Two hospital-based studies Patients with BMI (body mass index) > 25, which is showed that respiratory function was normal in the defined as overweight, did not report more symptoms majority of patients reporting shortness of breath, and than those of normal weight [10]. On the other hand, a cardiovascular deconditioning and being overweight recent weight gain was found to be a predictive factor for were the most common cause for this symptom [10, 75]. PPS [86]. Sleep disorders are common among PPS Respiratory impairment can occur without shortness patients [10], and can be a mix of obstructive sleep of breath and can present with daytime somnolence, apnoea, frequency of tiredness on waking up and during morning headache, and fatigue [67]. There are no ran- the day, headache on waking up, daytime sleepiness, domized trials evaluating the effect of respiratory aids. restless legs, and hypoventilation [87–89]. It is widely Reports indicate that early introduction of non-invasive accepted that obesity is related to obstructive sleep respiratory aids like intermittent positive pressure venti- apnoea, and weight control is crucial for this disorder lation (IPPV) or biphasic positive pressure (BIPAP) ven- [90]. The number of patients receiving mechanical home tilators via mouthpiece or nasal application can stabilize ventilation because of obesity-induced hypoventilation the situation and prevent complications such as chest has increased [91]. From this perspective, there is a ratio- infections, further respiratory decline, and invasive ven- nale for reducing excess weight in PPS patients (Class tilatory aid (tracheostomy) [73, 76], and also improve exercise capacity [77] (Class IV). If invasive ventilatory One pilot study reported that a change from metal aid is needed, PPS patients with a tracheostomy and braces to lightweight carbon orthoses can be useful mechanical home ventilation are reported to have good and increase walking ability in polio patients with new perceived health despite severe physical disability [78] pareses [92]. This has been confirmed in two other open (Class III). For patients already using intermittent respi- uncontrolled studies [93, 94], and there is Class III ratory aids, respiratory muscle training is useful [79] evidence that lightweight orthoses should be preferred (Class IV). General precautions such as stopping compared to metal braces. Biomechanical analysis of smoking, mobilization of secretions, and cough assis- the walking pattern can lead to optimal design of ortho- tance are beneficial [73].
ses and improve function in the lower limbs (Class IV) [94, 95].
Frequent periods of rest, energy conservation, and work simplification skills are thought to be useful for Weakening of bulbar muscles causing dysphagia, weak- patients with fatigue [96].
ness of voice, and vocal changes have been reported among patients with PPS [80–83]. Case reports indicate Coming to terms with new disabilities,
that speech therapy and laryngeal muscle training are useful for these patients (Class IV) [83].
New loss of function, increase in disability, and handicap are common in post-polio patients [5, 10, 97]. This can Weight control, assistive devices,
lead to reduced wellbeing and emotional stress. Group and lifestyle modifications
training with other post-polio patients, participation, The importance of reducing weight, adaptation to assis- and regular follow-up at post-polio clinics can prevent a tive devices, and modification of activities of daily living decline in mental status and give a more positive experi- has been emphasized [4, 6, 84, 85]. The scientific evi- ence of the ‘self' [71, 98] (Class III). Acceptance of assis- dence for these recommendations is limited, but there tive devices, environmental support, and spending more was consensus in our group that an individual with weak time on daily tasks can facilitate coping with home and muscles benefits from losing excess weight, and that occupational life (Class III) [99].
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long-term effects of muscular training. A potential posi- tive effect of IvIg in PPS has been claimed in three recent studies, and follow-up studies to investigate whether IvIg • Some controlled studies of potential specific medical could be a therapeutic option are needed.
treatments for PPS have been completed, and no definitive therapeutic effect has been reported for the agents pyridostigmine, steroids, amantadine, modafinil, and coenzyme Q10.
Conflicts of interest
Level B• Supervised muscular training, both isokinetic and The authors have reported no conflicts of interests.
isometric, is a safe and effective way to prevent further decline of muscle strength in slightly or moderately weak muscle groups and can even reduce symptoms of muscular fatigue, muscle weakness, and pain in selected post-polio patients. A prolonged effect up to one year after well-defined training programmes has been reported.
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• Precautions to avoid muscular overuse should be taken 3. Halstead LS, Rossi CD. New problems in old polio patients: with intermittent breaks, periods of rest between series of results of a survey of 539 polio survivors. Orthopedics exercises, and submaximal work load.
• Training in a warm climate and non-swimming water 4. MarchofDimes. March of Dimes International Conference exercises are particularly useful.
on Post Polio Syndrome. Identifying best practices in diag-nosis and care. In: •••• (ed.) March of Dimes, NY, USA: Level C• Recognition of respiratory impairment and early White Plains, 2000; pp. ••–••.
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