Guideline:
EFNS guideline on diagnosis and management of post-polio syndrome
Bibliographic Source(s)
- Farbu E Gilhus NE Barnes MP Borg K de Visser M Driessen A Howard R Nollet F Opara J Stalberg E. EFNS guideline on diagnosis and management of post-polio syndrome. Report of an EFNS task force. Eur J Neurol 2006 Aug;13(8):795-801. [80 references] PubMed
Guideline Status
This is the current release of the guideline.
There are ongoing studies evaluating the effect of immune modulating therapy in post-polio syndrome (PPS). The results will probably be ready within the next 2 years. A revision of these guidelines would be useful at the same time.
Guideline Category
Diagnosis
Management
Rehabilitation
Treatment
Intended Users
Physical Therapists
Physicians
Respiratory Care Practitioners
Guideline Objective(s)
To develop a common definition of post-polio syndrome (PPS) and evaluate the existing evidence for the clinical effectiveness of therapeutic interventions and on this basis provide clinical guidelines for management of PPS
Target Population
Patients with post-polio syndrome (PPS)
Interventions and Practices Considered
Diagnosis
Assessing symptoms and ruling out all other possible causes of new symptoms
Treatment/Management
- Supervised muscular training (isokinetic and isometric)
- Training in a warm climate non-swimming water exercises
- Respiratory muscle training
- Group training regular follow-up and patient education
- Weight loss
- Use of properly fitted assistive devices
Note: Pyridostigmine steroids and amantadine were considered but not recommended because of lack of therapeutic effect.
Major Outcomes Considered
Effectiveness of treatment in improving muscle strength and cardiovascular fitness and reducing pain
Methods Used to Collect/Select Evidence
Searches of Electronic Databases
Description of Methods used to Collect/Select the Evidence
Medline via Pubmed EMBASE ISI and the Cochrane Library were searched from 1966 to 2004. Search terms were PPS/post-poliomyelitis/PPMA/PPMD/poliomyelitis in combination with management therapy treatment medicaments physiotherapy and intervention.
No meta-analyses of interventions for post-polio syndrome (PPS) were found when searching the databases.
Number of Source Documents
Not stated
Methods Used to Assess the Quality and Strength of the Evidence
Weighting According to a Rating Scheme (Scheme Given)
Rating Scheme for the Strength of the Evidence
Evidence Classification Scheme for a Diagnostic Measure
Class I: A prospective study in a broad spectrum of persons with the suspected condition using a "gold standard" for case definition where the test is applied in a blinded evaluation and enabling the assessment of appropriate tests of diagnostic accuracy
Class II: A prospective study of a narrow spectrum of persons with the suspected condition or a well-designed retrospective study of a broad spectrum of persons with an established condition (by "gold standard") compared to a broad spectrum of controls where test is applied in a blinded evaluation and enabling the assessment of appropriate tests of diagnostic accuracy
Class III: Evidence provided by a retrospective study where either persons with the established condition or controls are of a narrow spectrum and where test is applied in a blinded evaluation
Class IV: Any design where test is not applied in blinded evaluation OR evidence provided by expert opinion alone or in descriptive case series (without controls)
Evidence Classification Scheme for a Therapeutic Intervention
Class I: An adequately powered prospective randomized controlled clinical trial with masked outcome assessment in a representative population or an adequately powered systematic review of prospective randomized controlled clinical trials with masked outcome assessment in representative populations. The following are required:
- Randomization concealment
- Primary outcome(s) is/are clearly defined
- Exclusion/inclusion criteria are clearly defined
- Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias
- Relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences
Class II: Prospective matched-group cohort study in a representative population with masked outcome assessment that meets a–e above or a randomized controlled trial in a representative population that lacks one criteria a–e
Class III: All other controlled trials (including well-defined natural history controls or patients serving as own controls) in a representative population where outcome assessment is independent of patient treatment
Class IV: Evidence from uncontrolled studies case series case reports or expert opinion
Methods Used to Analyze the Evidence
Systematic Review
Description of the Methods Used to Analyze the Evidence
Not stated
Methods Used to Formulate the Recommendations
Expert Consensus
Description of Methods Used to Formulate the Recommendations
Data were classified according to their scientific level of evidence as class I–IV. Recommendations are given as level A–C according to the scheme for European Federation of Neurological Societies (EFNS) guidelines (see the "Availability of Companion Documents" field in this summary). When only class IV evidence was available but consensus could be reached the Task Force gives recommendations as good practice points. Consensus was reached mainly through e-mail correspondence.
A questionnaire about diagnosis management and care of post-polio patients was answered by the group members from the Netherlands Norway Poland Sweden and UK.
Rating Scheme for the Strength of the Recommendations
Rating of Recommendations for a Diagnostic Measure
Level A rating (established as useful/predictive or not useful/predictive) requires at least one convincing class I study or at least two consistent convincing class II studies.
Level B rating (established as probably useful/predictive or not useful/predictive) requires at least one convincing class II study or overwhelming class III evidence.
Level C rating (established as possibly useful/predictive or not useful/predictive) requires at least two convincing class III studies.
Rating of Recommendations for a Therapeutic Intervention
Level A rating (established as effective ineffective or harmful) requires at least one convincing class I study or at least two consistent convincing class II studies.
Level B rating (probably effective ineffective or harmful) requires at least one convincing class II study or overwhelming class III evidence.
Level C rating (possibly effective ineffective or harmful) requires at least two convincing class III studies.
Good Practice Points When only class IV evidence was available but consensus could be reached the Task Force gives recommendations as good practice points.
Cost Analysis
A formal cost analysis was not performed and published cost analyses were not reviewed.
Method of Guideline Validation
Peer Review
Description of Method of Guideline Validation
The guidelines were validated according to the European Federation of Neurological Societies (EFNS) criteria (see "Availability of Companion Documents" field).
Major Recommendations
The levels of evidence (class I-IV) supporting the recommendations and ratings of recommendations (A-C Good Practice Points) are defined at the end of the "Major Recommendations" field.
Diagnostic Criteria
The Task Force suggests that the criteria for post-polio syndrome (PPS) used within European Federation of Neurological Societies (EFNS) and Europe should be based on the Halstead's definition from 1991 with emphasis on the new muscle weakness (refer to the original guideline document for details). 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.
Therapeutic Interventions
Level A Recommendations
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 steroids and amantadine).
Level B Recommendations
Supervised 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 selected post-polio patients. There are no studies evaluating the effect of muscular training in patients with severe weakness and the long-term effects of such training are not yet explored. Precautions to avoid muscular overuse should be taken with intermittent breaks periods of rest between series of exercises and submaximal work load.
Training in a warm climate and non-swimming water exercises are particularly useful.
Level C Recommendations
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.
Respiratory muscle training can improve pulmonary function.
Group training regular follow-ups and patient education are useful for the patients' mental status and well-being.
Good Practice Points
Weight loss and adjustment and introduction of properly fitted assistive devices; but lack significant scientific evidence.
Definitions:
Evidence Classification Scheme for a Diagnostic Measure
Class I: A prospective study in a broad spectrum of persons with the suspected condition using a "gold standard" for case definition where the test is applied in a blinded evaluation and enabling the assessment of appropriate tests of diagnostic accuracy
Class II: A prospective study of a narrow spectrum of persons with the suspected condition or a well-designed retrospective study of a broad spectrum of persons with an established condition (by "gold standard") compared to a broad spectrum of controls where test is applied in a blinded evaluation and enabling the assessment of appropriate tests of diagnostic accuracy
Class III: Evidence provided by a retrospective study where either persons with the established condition or controls are of a narrow spectrum and where test is applied in a blinded evaluation
Class IV: Any design where test is not applied in blinded evaluation OR evidence provided by expert opinion alone or in descriptive case series (without controls)
Evidence Classification Scheme for a Therapeutic Intervention
Class I: An adequately powered prospective randomized controlled clinical trial with masked outcome assessment in a representative population or an adequately powered systematic review of prospective randomized controlled clinical trials with masked outcome assessment in representative populations. The following are required:
- Randomization concealment
- Primary outcome(s) is/are clearly defined
- Exclusion/inclusion criteria are clearly defined
- Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias
- Relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences
Class II: Prospective matched-group cohort study in a representative population with masked outcome assessment that meets a–e above or a randomized controlled trial in a representative population that lacks one criteria a–e
Class III: All other controlled trials (including well-defined natural history controls or patients serving as own controls) in a representative population where outcome assessment is independent of patient treatment
Class IV: Evidence from uncontrolled studies case series case reports or expert opinion
Rating of Recommendations for a Diagnostic Measure
Level A rating (established as useful/predictive or not useful/predictive) requires at least one convincing class I study or at least two consistent convincing class II studies.
Level B rating (established as probably useful/predictive or not useful/predictive) requires at least one convincing class II study or overwhelming class III evidence.
Level C rating (established as possibly useful/predictive or not useful/predictive) requires at least two convincing class III studies.
Rating of Recommendations for a Therapeutic Intervention
Level A rating (established as effective ineffective or harmful) requires at least one convincing class I study or at least two consistent convincing class II studies.
Level B rating (probably effective ineffective or harmful) requires at least one convincing class II study or overwhelming class III evidence.
Level C rating (possibly effective ineffective or harmful) requires at least two convincing class III studies.
Good Practice Points When only class IV evidence was available but consensus could be reached the Task Force gives recommendations as good practice points.
Clinical Algorithm(s)
None provided
Type of Evidence supporting the Recommendations
The type of supporting evidence is identified and graded for selected recommendations (see "Major Recommendations" field).
Potential Benefits
Appropriate diagnosis and treatment of post-polio syndrome (PPS)
Potential Harms
Precautions to avoid muscular overuse while exercising should be taken with intermittent breaks periods of rest between series of exercises and submaximal work load.
Qualifying Statements
This guideline provides the view of an expert task force appointed by the Scientific Committee of the European Federation of Neurological Societies (EFNS). It represents a peer-reviewed statement of minimum desirable standards for the guidance of practice based on the best available evidence. It is not intended to have legally binding implications in individual cases.
Description of Implementation Strategy
The European Federation of Neurological Societies has a mailing list and all guideline papers go to national societies national ministries of health World Health Organisation European Union and a number of other destinations. Corporate support is recruited to buy large numbers of reprints of the guideline papers and permission is given to sponsoring companies to distribute the guideline papers from their commercial channels provided there is no advertising attached.
Implementation Tools
Staff Training/Competency Material
For information about availability see the "Availability of Companion Documents" and "Patient Resources" fields below.
IOM Care Need
Getting Better
Living with Illness
IOM Domain
Effectiveness
Patient-centeredness
Bibliographic Source(s)
- Farbu E Gilhus NE Barnes MP Borg K de Visser M Driessen A Howard R Nollet F Opara J Stalberg E. EFNS guideline on diagnosis and management of post-polio syndrome. Report of an EFNS task force. Eur J Neurol 2006 Aug;13(8):795-801. [80 references] PubMed
Adaptation
Not applicable: The guideline was not adapted from another source.
Source(s) of Funding
European Federation of Neurological Societies
Guideline Committee
European Federation of Neurological Societies Task Force on the Diagnosis and Management of Post-Polio Syndrome
Composition of Group that Authored the Guideline
Task Force Members: E. Farbu Department of Neurology Stavanger University Hospital Stavanger Norway; N. E. Gilhus Department of Neurology Haukeland University Hospital University of Bergen Bergen Norway; M. P. Barnes Academic Unit of Neurological Rehabilitation Hunters Moor Hospital Newcastle upon Tyne UK; K. Borg Department of Public Health Sciences Division of Rehabilitation Medicine Danderyds University Hospital Karolinska Intitutet Karolinska Hospital Stockholm Sweden; M. de Visser Department of Neurology Academic Medical Center University of Amsterdam Amsterdam The Netherlands; A. Driessen Lt. Gen. Van Heutzlaan Baarn The Netherlands; R. Howard Department of Neurology St Thomas' Hospital Lambeth Palace Road London UK; F. Nollet Department of Rehabilitation Medicine Academic Medical Center University of Amsterdam Amsterdam The Netherlands; J. Opara Repty Rehab Centre ul. Sniadeckio 1 Tarnowskie Góry Poland; E. Stalberg Department of Clinical Neurophysiology University Hospital Uppsala Sweden
Financial Disclosures/Conflicts of Interest
The authors have reported no conflicts of interests.
Guideline Status
This is the current release of the guideline.
There are ongoing studies evaluating the effect of immune modulating therapy in post-polio syndrome (PPS). The results will probably be ready within the next 2 years. A revision of these guidelines would be useful at the same time.
Guideline Availability
Electronic copies: Available to registered users from the European Federation of Neurological Societies Web site.
Print copies: Available from E. Farbu Department of Neurology Stavanger University Hospital N-4068 Stavanger Norway; Phone: +47 5151 8447; Fax: +47 5151 9916; E-mail: elfa@sir.no
Availability of Companion Documents
The following are available:
- Brainin M Barnes M Baron JC Gilhus NE Hughes R Selmaj K Waldemar G; Guideline Standards Subcommittee of the EFNS Scientific Committee. Guidance for the preparation of neurological management guidelines by EFNS scientific task forces – revised recommendations 2004. Eur J Neurol. 2004 Sep;11(9):577-81. Electronic copies: Available in Portable Document Format (PDF) from the European Federation of Neurological Societies Web site.
- Guideline papers. European Federation of Neurological Societies. Electronic copies: Available from the European Federation of Neurological Societies Web site.
- Continuing Medical Education questions available from the European Journal of Neurology Web site.
Patient Resources
None available
NGC STATUS
This NGC summary was completed by ECRI on April 9 2007. The information was verified by the guideline developer on May 15 2007.
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This NGC summary is based on the original guideline which is subject to the Blackwell-Synergy copyright restrictions.
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