PSN Library Files

EFNS Guideline on diagnosis and management of post-polio syndrome.

Report of an EFNS Task Force on Post-polio Syndrome.

Farbu E’, Barnes MP, Driessen A?, Borg K4, de Visser M, Howard R”, Nollet F1, Opara Jo, Stalberg E”, Gilhus NE

‘Dept. of Neurology, Haukeland University Hospital, University of Bergen, N 5021 Bergen, Norway, elisabeth,farbu@helse-bergen.no

-Academic Unit of Neurological Rehabilitation, Hunters Moor Hospital, Newcastle upon Tyne NE2 4NR, UK

Lt. Gen. Van Heutzlaan 6, 3743 JN Baarn, The Netherlands

“Division of Rehabilitation Medicine, Department of Public Health Sciences, Karolinska Intitutet/Karolinska Hospital, S-171 76 Stochkholm Sweden

Dept. of Neurology, Academic Medical Center, University of Amsterdam, 1100 DD Amsterdam, The Netherlands

Dept. of Neurology, St. Thomas Hospital, Lambeth Palace Road, London SE1 7EH, UK

Dept. of Rehabilitation Medicine, Academic Medical Center, University of Amsterdam, 1100 DD Amsterdam, The Netherlands

8Repty Rehab Centre, ul. Sniadeckio 1, PL 42-604 Tarnowskie Góry, Poland

Dept. of Clinical Neurophysiology, University Hospital, S-75185 Uppsala, Sweden

Correspondence to: E. Farbu

Abstract

Post-polio syndrome (PPS) is characterised by new or increased muscular

weakness, atrophy, muscle pain and fatigue several years after acute polio. PPS is

a clinical diagnosis with no specific tests, and a careful investigation is needed to

rule out other possible causes for the new symptoms. The Task Force suggests

exact criteria for PPS.

When evaluating therapeutic interventions, we find that muscular training, both

isokinetic and isometric is a safe and effective way to prevent further decline for

patients with moderate weakness, and can be recommended at 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. At

level B we also recommend respiratory muscle training, which 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 of invasive respiratory aids. Group training, regular follow-ups, and

patient education are useful for the patients’ mental status and well being.

Weight loss, adjustment and introduction of properly fitted assistive devices are

regarded as good practice points, but lack proper scientific evidence. Steroids and

acetylcholinesterase inhibitors have no place in the treatment of PPS.

As there is limited data about effects and treatment of pain, future randomised

studies of therapeutic interventions should particularly address the potential effect

on this symptom, which is commonly reported by PPS patients. There is also a

need for studies evaluating the long-term effects of muscular training.

Objectives

The number of new cases of acute paralytic polio has fallen rapidly during the last

decade due to vaccination campaigns. Still, there are several patients with

previous paralytic polio worldwide, experiencing new symptoms and functional

decline later in life. The term post-post polio syndrome (PPS) has been used to

cover many of the symptoms reported by previous polio patients (Dalakas 1995;

Borg 1996; Halstead and Rossi 1985). A Task Force on Post-polio syndrome

under the auspices of the European Federation of Neurological Societies (EFNS)

was set up in 2002. The aim was to make a common definition of PPS and

evaluate the existing evidence for the clinical effectiveness of therapeutic

interventions and on this basis provide clinical guidelines for management of PPS.

Background

Many previous polio patients experience new muscle weakness, new atrophy,

fatigue, muscular and joint pain, and cold intolerance several years after acute

paralytic poliomyelitis. A case of new atrophy and weakness several years after

acute paralytic polio was first described in 1875 by Raymond (Raymond 1875).

The term post-polio syndrome (PPS) was introduced by Halstead in 1985 to cover

medical, orthopaedic, and psychological problems possibly or indirectly related to

the long term disability occurring many years after the acute episode. The criteria

for PPS were:

1) Confirmed history of polio

2) Partial or fairly complete neurological and functional recovery after

the acute episode

3) Period of at least 15 years with neurological and functional stability

4) Two or more of the following health problems occurring after the

stable period: – extensive fatigue, muscle and/or joint pain, new

weakness in muscles previously affected or unaffected, new muscle

atrophy, functional loss, cold intolerance

5) No other medical explanation found (Halstead and Rossi 1985).

Halstead revised these criteria in 1991 and added “gradual or abrupt onset of new

neurogenic weakness” as an necessary criterion for PPS, with or without other co

existing symptoms (Halstead 1991).

Dalakas redefined and narrowed the use of PPS in 1995. He combined the criteria

for post-polio muscular atrophy (PPMA), i.e. new muscular atrophy at least 15

years after the acute infection, and the following symptoms: fatigue and decreased

endurance, increase in skeletal deformities, and pain in joints (Dalakas 1995). A

third term, post-polio muscular dysfunction (PPMD), was introduced in 1996 with

the following criteria:

1) History of paralytic polio, confirmed or not confirmed; partial or fairly

complete functional recovery

2) After a period of functional stability of at least 15 years development

of new muscle dysfunction: muscle weakness, muscle atrophy, muscle

pain, fatigue

3) Neurological examination compatible with prior polio: lower motor

neurone lesion; decreased or absent tendon reflexes; no sensory loss;

compatible findings on EMG and/or MRI (Borg 1996).

The symptoms reported for PPS are the same from all parts of the world. Muscle

weakness, atrophy, generalised fatigue, post-exercise fatigue, muscle pain,

fasciculations, cramps, cold intolerance, and joint pain dominate (Jubelt and Agre

2000; Ivanyi et al. 1999; Farbu and Gilhus 2001; Farbu and Gilhus 2002; Rekand

et al. 2004; Halstead and Rossi 1985; Farbu et al. 2003; Chang and Huang 2001;

Takemura et al. 2004). A history of previous paralytic polio seems to increase

long term mortality (Nielsen et al. 2003).

The prevalence of PPS has been reported from 15-80% of all patients with

previous polio depending on the criteria applied and population studied (Farbu et

1. 2003;Ivanyi et al. 1999;Burger and Marincek 2000;Dalakas 1995;Halstead and

Rossi 1987;Halstead 1998; Ramlow et al. 1992).

For symptomatic treatment and clinical purposes the difference between stable

muscle weakness after polio and PPS often remains insignificant. Still, it would

be of great benefit to have a consensus on the term PPS, both for clinical use, but

also for research. All three definitions are based on the principle of exclusion of

other causes for new deterioration and symptoms. Halstead claimed that two

different symptoms, like joint pain and cold intolerance, was sufficient for a

diagnosis of PPS, but redefined and included new muscle weakness as an

obligatory criterion for the diagnosis. Dalakas proposed an even more focused

neuromuscular approach where new atrophy was the cornerstone. Still, many

patients report a sense of weakening in the muscles before it is detectable by

clinical examination as new atrophy. These findings can be confirmed by

isometric muscle strength evaluation and computer tomography imaging (Ivanyi

et al. 1998;Lygren et al. 2004). Atrophy is the end stage of new neuromuscular

deterioration and by using this as an necessary criterion, patients in an earlier

stage of neuromuscular deterioration will be excluded. In principle, the revised

Halstead criteria and Borg’s PPMD definition give the same description of PPS

We suggest that the criteria for PPS used within EFNS and Europe should be

based on these two definitions with emphasis on the new muscle weakness. The

diagnosis of PPS is an exclusion diagnosis with no test or analysis specific for

PPS, and the role of the investigation is to rule out every other possible cause for

the new symptoms and clinical deterioration.

Role of clinical neurophysiology

These techniques are used for four main reasons. First, to establish a picture

typical of lower motor neurone involvement (neurogenic EMG findings, normal

sensory findings, and normal motor findings except for parameters reflecting

muscle atrophy). Second, to exclude other causes of the symptoms. This is part of

the PPS definition, and it is not uncommon to find patients in whom the initial

diagnosis of polio must be revised. Third, to find concomitant nerve or muscle

disorders, such as entrapments and radiculopathies. Fourth, to assess the degree of

motor neurone loss. This cannot be quantified clinically, since loss of neurones

may be completely masked by compensatory nerve sprouting and muscle fibre

hypertrophy. Macro EMG studies have shown that loss of up to 50% of neurones

may be compatible with a normal clinical picture (Stalberg and Grimby 1995).

In longitudinal studies with macro EMG a continuous loss of neurones takes

place with exaggerated speed compared to normal age dependent degeneration

(Grimby et al. 1998). New weakness appears when the compensatory mechanisms

are no longer sufficient, and occurs when less than 10-20% of the neurones

remain.

Search strategy

Medline via Pubmed, EMBASE, ISI, and Cochrane were searched with time

limits 1966 until present. Search terms were post-polio

syndrome/postpoliomyelitis/PPMA/PPMD/poliomyelitis in combination with

management, therapy, treatment, medicaments, physiotherapy, and intervention.

No meta-analyses of intervention in PPS were found when searching the

databases.

Data were classified according to their scientific level of evidence (Brainin et al.

2004)

The classification for evidence levels for health care interventions are the

following:

la: Evidence obtained from meta-analysis of randomised controlled trials

Ib: Evidence obtained from at least one randomised controlled trial

Ila: Evidence obtained from at least one well-designed, controlled, but not

randomised study

IIb: Evidence obtained from at least one other type of well-designed quasi

experimental study in which implementation of an intervention is outside the

control of the investigators, but an opportunity exists to evaluate its effect)

III: Evidence obtained from well-designed, non-experimental descriptive studies,

such as comparative studies, correlation studies and case studies

IV: Evidence obtained from expert committee reports or opinions and/or clinical

experience of respected authorities

The classification of grades for recommendations are:

A: Requires at least one randomised controlled trial as part of a body literature of

overall good quality and consistency addressing the specific recommendation

(Evidence level la, lb)

B: Requires the availability of well conducted studies but not randomised

evidence on the topic of the recommendation (Evidence level IIa, IIb, III)

C: Requires the evidence obtained from expert committee reports or opinions

and/or clinical experiences of respected authorities. Indicates an absence of

directly applicable studies of good quality (Evidence level IV)

A survey of questions about diagnosis, management, and care of post-polio

patients was answered by the group members from five different European

countries.

Results

National surveys

None of the nations represented in this task force had formal national guidelines

for PPS, neither for diagnosis nor treatment. Diagnostic criteria applied were

those of Halstead (Sweden), Borg (the Netherlands), and Dalakas (Norway).

There were no national competence centres in any of the countries. Medical

specialities involved were mainly physical medicine and rehabilitation, neurology,

clinical neurophysiology, respiratory medicine, and orthopaedics. Neurologists

were involved in diagnosis whereas rehabilitation physicians were involved in

long term management and care. In UK, PPS patients were mainly taken care of

by their general practitioners with less contact with the secondary level of the

health service.

Therapeutic interventions

Acetylcholinesterase inhibitors, steroids, and amantadine

The effect of pyridostigmine in PPS has been investigated in four studies with

particular emphasis on the aspects fatigue, muscular strength, and quality of life.

Two open pilot studies indicated a positive effect on fatigue (Trojan and Cashman

1995), but this was not confirmed in two double-blinded randomised controlled

trials (Horemans et al. 2003;Trojan et al. 1999). Horemans et al. reported a

significant improvement in walking performance, but the difference in quadriceps

strength was not significant as reported by Trojan et al. Hence, there is evidence

at level Ib that pyridostigmine is not effective in the management of fatigue and

muscular strength in PPS. There are two randomised placebo controlled studies

investigating the effect of high dose prednisolone and amantandine on muscular

strength and muscular fatigue (Dinsmore et al. 1995; Stein et al. 1995). Both were

negative with no symptom improvement.

Muscular training

It has been claimed that muscular overuse and training may worsen the symptoms

in PPS and even provoke a further loss of muscular strength. (Bennett and

Knowlton 1958). Many post-polio patients have been advised to avoid muscular

overuse and intensive training (March of Dimes 2000; Halstead and Gawne

1996). Studies of muscle morphology and oxidative capacity in the tibialis

anterior muscle indicate a high muscular activity due to gait and weight bearing

(Borg and Henriksson 1991; Grimby et al. 1996). Macro MUP (macro EMG

motor unit potential) amplitude in the tibialis anterior muscle was found to be

increased after five years, whereas there was no change in the Macro MUP

amplitude in the biceps brachii muscle (Sandberg and Stalberg 2004). This

indicates a more pronounced denervation-reinnervation process in the tibialis

muscle compared to the biceps muscle and it is speculated whether this is due to

daily use and higher muscular activities in the leg muscles. However, none of

these studies have assessed the levels of activity. There are no prospective studies,

which show that increased muscular activity or muscle training lead to loss of

muscular strength compared to absence of training or less muscular activity. On

the contrary, patients who reported regular physical activity had less symptoms

and a higher functional level than physically inactive patients (Veicsteinas et al.

1998; Rekand et al. 2004). One randomised controlled trial reported significant

improvement in muscular strength after a 12 week training programme with

isometric contraction of hand muscles (Chan et al. 2003). Non-randomised trials

with training programmes lasting from six weeks to six months involving both

isokinetic, isometric and endurance muscular training have shown a significant

increase in both isokinetic and isometric muscle strength (Agre et al. 1997;

Einarsson 1991; Ernstoff et al. 1996; Spector et al. 1996). No complications or

side effects were reported. Hence, there is evidence at level Ib, also supported by

several trials at level IIb, that supervised training programmes increase muscle

strength in patients with post-polio syndrome. It should be added that the long

term effects (years) of training are not documented, but deserve more studies.

Aerobic walking exercises can help to economise movements and increase

endurance without improvement in cardiovascular fitness (Dean and Ross 1991).

Ernstoff et al. reported an increase in work performance by reduction of heart rate

during exercises, hence endurance training seems to improve cardiovascular

conditioning (level Ilb). One randomised controlled study of post-polio patients

with pain, weakness, and fatigue in their shoulder muscles compared the effect of

exercise only, exercise in combination with lifestyle modification, and lifestyle

modification only (Klein et al. 2002). All three groups improved after

intervention, but significant difference only for the groups with exercise. The

NO

endpoints in this study were combinations and addition of several symptoms.

Thus further studies are needed to identify improvement on particular symptoms

before conclusions are drawn regarding lifestyle modifications.

Treatment in a warm climate and training in water

Anecdotal reports from post-polio patients indicate a positive effect of a warm

climate and of training in warm water with respect to pain and fatigue. One

randomised controlled study reported a significant reduction in pain, health

related problems and depression for both groups after completing identical

training programmes in either Norway or Tenerife (Strumse et al. 2003). No

significant difference in walking tests was seen. Both groups improved their

walking skills, reduced their level of fatigue, depression, and health-related

problems. However, the effect remained significantly longer in the Tenerife

group.

Dynamic non-swimming water exercises for post-polio patients have been

reported to reduce pain, improve cardiovascular conditioning, and increase

subjective well-being in a controlled but not randomised study (level Ila) (Willen

et al. 2001). A qualitative interview study (level III) indicates a positive effect on

the self experience when performing group training in water (Willen and

Scherman 2002).

Respiratory aid

Reduced pulmonary function due to weak respiratory muscles and/or chest

deformities may occur in patients with previous polio (Howard et al. 1988; Kidd

et al. 1997). Patients with chest deformities have an increased risk of nocturnal

hypoventilation and sleep-disordered breathing. The prevalence of respiratory

impairment is highest among patients who were treated with artificial ventilation

in the acute phase (Howard et al. 1988). Shortness of breath is a common

complaint in many post-polio patients, but is not necessarily related to respiratory

impairment. Two hospital based studies reported that respiratory function was

normal in the majority of patients reporting shortness of breath, and

cardiovascular deconditioning and being overweight were the most common

cause for this symptom (Farbu et al. 2003; Stanghelle et al. 1993). Respiratory

impairment can occur without shortness of breath and can present with daytime

somnolence, morning headache, and fatigue (Dean et al. 1991). There are no

randomised trials evaluating the effect of respiratory aids. Reports indicate that

early introduction of non-invasive respiratory aids like intermittent positive

pressure ventilation (IPPV) or biphasic positive pressure (BIPAP) ventilators via

mouthpiece or nasal application can stabilise the situation and prevent

complications like chest infections, further respiratory decline and invasive

ventilatory aid (tracheostomy) (Bergholtz et al. 1988; Bach 1995), and also

improve exercise capacity (Vaz Fragoso et al. 1992)(evidence at level III). If

invasive ventilatory aid is needed, PPS patients with a tracheostomy and

mechanical home ventilation are reported to have good perceived health despite

severe physical disability (Markstrom et al. 2002)(level III). For patients already

using intermittent respiratory aids, respiratory muscle training is useful (Klefbeck

et al. 2000)(level IIb). General precautions like stopping smoking, mobilisation of

secretions, and cough assistance are beneficial (Bergholtz et al. 1988).

Bulbar symptoms

Weakening of bulbar muscles causing dysphagia, weakness of voice, and vocal

changes have been reported among patients with PPS (Abaza et al. 2001; Driscoll

et al. 1995; Sonies and Dalakas 1991; Ivanyi et al. 1994). Case reports indicate

that speech therapy and laryngeal muscle training are useful for these patients

(evidence at level III) (Abaza et al. 2001).

Weight control, assistive devices, and lifestyle modifications

The importance of reducing weight, adaptation to assistive devices and

modification of activities of daily living have been emphasised by many

authorities in this field (Halstead et al. 1995; Jubelt and Agre 2000; Thorsteinsson

1997; March of Dimes 2000). The scientific evidence for these recommendations

is limited, but there was consensus in our group that an individual with weak

muscles benefits from losing excess weight, and that proper orthoses, walking

sticks, and wheelchairs facilitate daily life activities. Participating in muscle

training programmes and endurance training will, in many cases, also lead to

weight loss, but there is no evidence that weight reduction alone can ameliorate

symptoms. Patients with BMI (body mass index) > 25 which is defined as

overweight, did not report more symptoms than those of normal weight (Farbu et

1. 2003). However, sleep disorders are common amongst PPS patients (Farbu et
2. 2003), and can be a mix of obstructive sleep apnoea, frequency of tiredness on

waking up and during the day, headache on waking up, daytime sleepiness,

restless legs, and hypoventilation (Hsu and Staats 1998; Steljes et al. 1990;Van Kralingen et al. 1996). It is widely accepted that obesity is related to obstructive

sleep apnoea, and weight control is crucial for this disorder (Gami et al. 2003).

The number of patients receiving mechanical home ventilation because of

obesity-induced hypoventilation has increased (Janssens et al. 2003). From this

perspective, there is a rationale for reducing excess weight in PPS patients (level

III).

One pilot study reported that a change from metal braces to light weight carbon

orthoses can be useful and increase walking ability in polio patients with new

pareses (Heim et al. 1997). Biomechanical analysis of the walking pattern can

lead to optimal design of orthoses and improve function in the lower limbs (level

1. IV) (Perry and Clark 1997).

Frequent periods of rest, energy conservation, and work simplification skills are

thought to be useful for patients with fatigue who are not used to such techniques

in advance (Packer et al. 1991).

Coming to terms with new disabilities, educational interventions

New loss of function, increase in disability and handicap are common in post

polio patients (Ivanyi et al. 1999; Farbu et al. 2003; Nollet et al. 1999). This can

lead to reduced well-being and emotional stress. Group training with other post

polio patients, participation and regular follow-up at post-polio clinics can prevent

a decline in mental status and give a more positive experience of the “self”

(Stanghelle and Festvag 1997; Willen and Scherman 2002)(level III). Acceptance

of assistive devices, environmental support and spending more time on daily tasks

can facilitate with coping of home and occupational life (level III) (Thoren

Jonsson 2001).

Recommendations

Level A:

Steroids and acetylcholinesterase inhibitors have no place in the treatment of post

polio patients.

Level B:

Muscular training, both isokinetic and 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 post-polio patients. There are no studies exploring the effect of muscular

training in patients with severe weakness and the long-term effects of such

training is not yet explored.

Training in a warm climate and non-swimming water exercises are particularly

useful.

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 of

invasive respiratory aids.

Group training, regular follow-ups, and patient education are useful for the

patients’ mental status and well-being.

Level C:

Weight loss, and adjustment and introduction of properly fitted assistive devices

are good practice points, but lack significant scientific evidence.

Time for new revision of guidelines

There are now ongoing studies evaluating the effect of immune modulating

therapy in post-polio syndrome (Gonzalez et al. 2004). The results will probably

be ready within the next two years. A revision of these guidelines would be useful

at the same time. Prospective follow-up studies evaluating muscle strength and

function during the natural course of the disorder is welcomed. Studies evaluating

the effects of muscular training in patients with severe muscular weakness are

needed, in addition to prospective studies evaluating the long-term effects of

muscular training. Further randomised studies evaluating therapeutic interventions

should be performed with particular emphasis on pain and fatigue as these are

common and disabling symptoms and there is limited evidence that any

intervention can effect these symptoms.

Conflicts of interests

There are no conflicts of interests in this paper.

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