Elsevier

The Lancet Neurology

Volume 9, Issue 6, June 2010, Pages 634-642
The Lancet Neurology

Review
Management of postpolio syndrome

https://doi.org/10.1016/S1474-4422(10)70095-8Get rights and content

Summary

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

Introduction

12–20 million people worldwide have sequelae of poliomyelitis, according to Post-Polio Health International. Postpolio syndrome is the most common neuromuscular disorder in Sweden1 and the most prevalent motor neuron disease in the USA.2

A common misconception is that people in developed countries who have had poliomyelitis are old. Large epidemics were frequent in Europe and in the USA in the 1940s and during the first half of the 1950s when vaccination programmes were launched,2, 3 and many people who have recovered from poliomyelitis infection are still of working age. A global eradication of poliomyelitis is close to accomplishment; in a few years time there should be no new cases of the disorder. However, in several developing countries, many young people have recently contracted the disease. Although the number of people with sequelae of poliomyelitis will decrease in Europe and the USA in the next few decades, many people in developing countries will be affected for at least a generation.

Many health-care workers believe that the sequelae of poliomyelitis do not change throughout a patient's lifetime. However, more than a century ago the development of new or exacerbated symptoms was already reported long after the acute poliomyelitis infection.4 The existence of postpolio syndrome has been questioned, but the late effect of poliomyelitis, or postpolio syndrome, is generally accepted as a defined clinical entity.2, 5, 6 The prevalence of postpolio syndrome has been reported to be between 20% and 85% of people who have had poliomyelitis.2, 6, 7 This disparity is most probably caused by the use of different clinical diagnostic criteria. In this context, it is important to remember that people who have sequelae of poliomyelitis but who do not fulfil diagnostic criteria for postpolio syndrome might still have substantial loss of motor function and be in need of therapeutic interventions.

Interest in postpolio syndrome has increased over the past two to three decades, with research varying in focus from molecular to clinical aspects, and health-related quality of life. In this Review, we provide a comprehensive summary of the pathophysiology and clinical characteristics of postpolio syndrome, outline diagnostic and treatment options, and suggest future research strategies.

Section snippets

Pathophysiology

Studies of motor units of patients with postpolio syndrome have revealed an ongoing denervation–reinnervation process.8, 9 This is probably initiated after the acute poliomyelitis, and over time leads to increased motor unit areas caused by collateral sprouting of adjacent motor neurons in the spinal cord in patients with postpolio syndrome; a process also evident during normal ageing, although not until the seventh decade of life.10 The motor unit area might increase by up to 20 times,

Diagnosis

Several diagnostic criteria for postpolio syndrome have been proposed.8, 34, 35, 36 Different criteria from different countries focus on muscle atrophy34 or muscle dysfunction,10 but all are mainly based on those originally suggested by Halstead and Rossi.36 In 1991, Halstead added gradual or abrupt onset of new neurogenic muscular weakness as a criterion.37 In the criteria suggested by the 29th European Neuromuscular Centre workshop on postpolio muscle dysfunction, clinical and

Symptoms and signs

Because poliomyelitis affects the anterior horn cells in the anterior part of the spinal cord (the first part of the motor unit), postpolio syndrome is a lower motor neuron disorder. The symptoms in patients with postpolio syndrome are muscle weakness and atrophy, and fatigue and pain from muscles and joints (table 1).2, 37 Other symptoms include cold intolerance, difficulty swallowing, muscle twitching and cramps, respiratory distress, and voice changes.2, 3, 7, 17, 37

On clinical

Clinical management

No specific treatment for postpolio syndrome exists. Evaluation of the efficacy of treatment regimens for patients with postpolio syndrome was initiated by the Cochrane Library in 2009. Patients with postpolio syndrome often have a wide range of medical problems and may also have associated problems with participation in society, such as need for technical aids at home and at work.

Future perspectives

Better distinction between postpolio syndrome and poliomyelitis-related secondary disorders by more thorough clinical analyses is important. Further studies are needed to establish if there are clinical markers or risk factors that might predict the development of postpolio syndrome. If identified, measures for preventing postpolio syndrome could be started at an early stage. Further study of subgroups of patients with postpolio syndrome (eg, assessing rate of progression of muscle weakness and

Search strategy and selection criteria

References for this Review were identified through searches of The Cochrane Library (1950 to April, 2010), Medline (1950 to April, 2010), Embase (1974 to May, 2009), the Allied and Complementary Medicine Database (1985 to April, 2010), Cinahl (1984 to April, 2010), and Web of Science (1945 to April, 2010) with the search terms “post-poliomyelitis syndrome”, “poliomyelitis survivor”, “late onset poliomyelitis”, “late effect poliomyelitis”, “post-poliomyelitis”, “complications after

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