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Post-polio syndrome

Introduction

  • Post polio syndrome (PPS) is a neurological condition that can occur in people who have had polio. After an interval of several years of stability, people may then develop increasing weakness, stamina problems, fatigue and pain. PPS may respond to a range of therapies which might prevent further deterioration
  • Symptoms include the onset of new weakness or abnormal fatigue in previously affected or unaffected muscles; a general reduction in stamina; muscle and/or joint pain; muscle atrophy; breathing, sleeping and/or swallowing problems; or cold intolerance. Symptoms may lead to loss of endurance or function
  • A diagnosis of PPS assumes the absence of any other conditions that could explain the above symptoms. Such conditions should be considered and excluded by appropriate investigations

Diagnosis

  • As there is no diagnostic test for PPS, it is commonly defined by a symptom complex that includes new muscle weakness, decreased endurance, pain and fatigue
  • The onset of PPS may be gradual or it can occur suddenly. It occurs irrespective of ageing. Symptoms may sometimes appear to be triggered by various events like surgery, falls or immobility
  • The following are the main criteria that need to be considered in making a diagnosis of clinically definite PPS or clinically possible PPS

Clinically definite PPS

  • Based on various consensus statements, a diagnosis of clinically definite PPS comprises a confirmed history and/or physical evidence (however slight) of polio, a period of functional recovery and stability, new muscle weakness, or abnormal muscle fatigue,with evidence of neurogenic change, and the exclusion of any other possible conditions

Clinically possible PPS

  • It is recognised that there are patients who have PPS symptoms, but there is less diagnostic certainty. This would include patients with the following characteristics–a possible history of polio where there may be no previous physical manifestation, new muscle weakness, or abnormal muscle fatigue, (with no evidence of neurogenic change), a complex of symptoms that are generally recognised to be those for PPS, and the exclusion of any other possible conditions
  1. Definite history of polio/physical evidence. The patient’s original medical records, history and/or physical evidence provide a confirmed diagnosis and history of the original polio illness
  2. History of possible polio. Some people may not have confirmation of prior polio or a physical manifestation of the illness, but do have some history and/or current symptoms, which taken together, indicate a possibility of polio
  3. Period of recovery and stability. Partial or fairly complete neurological and functional recovery after the original polio illness followed by a period of neurological and functional stability. As guidance only, the period of stability will generally be 15 years or more
  4. New muscle weakness—with evidence of neurogenic change. Muscle weakness may be confirmed clinically by the presence of clear lower motor neuron features. When and if available/appropriate, EMG testing may confirm this and establish a baseline for repetitive testing or offer an alternative diagnosis. Other tests that may be useful are nerve conduction tests to assess nerve damage, manual muscle testing (MMT), reflex and exercise testing for endurance. Other causes of neuromuscular weakness will also need to be excluded by appropriate tests
  • Although it is accepted that electro-diagnostic testing has limitations in confirming neurogenic weakness, and will not provide a definitive diagnosis of new weakness, it may help to exclude some of the other common causes of neurogenic weakness as well as other, more rare, conditions
  1. New muscle weakness – no evidence of neurogenic change. A patient who has a possible history of polio may be experiencing new weakness that testing cannot confirm to be neurogenic (see Point 4) but is consistent with the symptoms of PPS
  2. Appropriate complex of symptoms. These may include two or more of the following health problems occurring after the stable period: extensive general fatigue, abnormal muscle fatigue, decreased endurance, muscle pain, joint pain, new weakness in muscles previously affected or unaffected, new muscle atrophy, functional loss, breathing or swallowing problems, cold intolerance
  3. No other disorder/medical explanation. Exclusion of medical, orthopaedic, and known neurological conditions that might cause the health problems listed in Point 6 above, although these other conditions may coexist with PPS. Depending on symptoms, the most obvious possible causes to rule out are orthopaedic problems related to the original polio; injuries, breathing problems, other neuromuscular diseases, and other diseases that commonly cause fatigue, such as thyroid problems, diabetes or heart disease
  • A diagnostic pathway for Post-polio syndrome (PPS)—suspected” is available on the Map of Medicine Healthguides via NHS Choices or www.mapofmedicine.com/access-map
Diagnostic criteria for clinically definite PPS and clinically possible PPS
Diagnosis criteriaClinically definite PPSClinically possible PPS
1. Definite history/physical evidence of polio  
2. History of possible polio  
3. Period of recovery and stability
4. New muscle weaknesses—with evidence of neurogenic change  
5. New muscle weakness—no evidence of neurogenic change  
6. Appropriate complex of symptoms
7. No other disorder/medical explanation

Main symptoms

  • The following are considered to be the most common health problems resulting from PPS

Weakness

  • New or increasing weakness is often seen as the most recognisable symptom of PPS. It can sometimes be difficult to separate weakness from muscle fatigue, which could be thought of as ‘weakness that develops over time’ or through usage
  • Recent definitions of PPS usually include both weakness and muscle fatigue (also known as peripheral fatigue, or stamina or endurance problems) as essential symptoms. Both symptoms can occur not only in muscles previously known to be affected by polio, but also in muscles where no damage has been apparent until now

Fatigue

  • Post polio fatigue is often experienced as two separate types of fatigue: a general, sometimes overwhelming, exhaustion (which may include mental fatigue), and localised muscle fatigue, often described as increasing physical weakness, loss of strength and endurance during exercise, and a heavy sensation in the muscles
  • Muscle fatigue after even minimal exercise can lead both to increasing weakness and the aching that is common in PPS. It may lead to general exhaustion or fatigue at the end of the day or it may even last for several days. Muscle fatigue can be a result of the muscle overuse that is thought to play a large part in PPS
  • General fatigue is an overwhelming feeling of exhaustion and weakness. Sometimes mental fatigue can be the result of muscle fatigue and muscle overuse. Fatigue can also result from sleep disturbance, sleep apnoea (stopping breathing for intervals) or breathing problems
  • Rest usually relieves fatigue, unless it has been building up for days or more, when it may take longer to feel better
  • General fatigue has been found in around half of people with polio, increasing to 80% for fatigue during exercise

Muscle pain

  • Muscle pain is very common and is usually described as aching, especially after activity, or felt as burning pain, spasms or cramps. This pain may be the result of muscle overuse and may occur with twitching or fasciculations, especially later in the day or at night
  • Research has shown pain can relate to activity levels, and particularly the intensity of activities, which is usually higher in people who had polio as they are using their muscles at their maximum much of the time

Joint pain

  • Where joints are no longer held in place by strong muscles, they may become unstable, resulting in joint pain. It may also be caused by injuries to the tendons or ligaments due to overuse of unstable joints. Weakness and injuries around joints may also lead to pain due to compressed (pinched) nerves

Muscle loss

  • Also known as muscle atrophy, this loss of muscle bulk has been found in 20–30% of people with PPS

Sleep disturbance

  • Sleep disturbances are common and may relate to sleep apnoea, breathing difficulties, pain, muscle twitching or general overtiredness. Sleep apnoea can be due to repeated shutting of the airway from throat weakness, sometimes made worse by weight gain. It can also occur when there are brief interruptions in the brain reflex that triggers breathing

Breathing problems

  • Breathing problems are more common in people who needed help with their breathing when they had polio, but can occur later on in anyone with polio. They are caused by muscle weakness of the diaphragm and chest muscles, scoliosis or sleep apnoea
  • Breathing problems may develop very slowly. Early signs may be frequent waking from sleep, sometimes with choking or gasping; nightmares, snoring, morning headaches, coughing, daytime sleepiness, difficulty speaking at length, lack of concentration and breathlessness with exercise

Swallowing problems (dysphagia)

  • Weakness in the muscles used for chewing and swallowing can cause coughing and less frequently choking and gagging. There may also be voice and speech changes, such as hoarseness or a nasal sounding voice, especially after speaking for a while or when tired
  • Most often, swallowing problems stay mild and progress only very slowly. Advice from a speech and language therapist may prove helpful

Cold intolerance

  • Sensitivity to cold, like many PPS symptoms, may be felt in one area of the body for example in an arm or leg which may have a poor blood supply, or may be felt as a general cold intolerance, or intolerance to any large temperature change

Other symptoms

  • There are many other symptoms that may or may not be related to polio. When other possible causes are ruled out, PPS is sometimes thought to be the cause. These symptoms may include urinary problems related to new pelvic floor weakness and/or detrusor denervation

Management

  • There is no cure for PPS. The effects of polio and PPS on each person vary. Delay in diagnosis may lead to further deterioration than is necessary. Management of PPS involves appropriate referral for multidisciplinary assessment from healthcare professionals, ideally with knowledge of polio/PPS together with use of self-management strategies
  • Energy management: over-activity or under-activity (boom or bust cycle) can lead to fatigue, increased weakness and pain. Although it is important to keep mobile, activity levels need to be balanced and paced, balancing rest and activity. Each person is an individual and professional guidance is recommended
  • Advice on general health: eating a healthy, well-balanced diet, weight loss if appropriate, not smoking and keeping warm enough are all important
  • Review use of aids and equipment: equipment and adaptive strategies may enable a person to be as active as possible whilst managing their energy levels. Some people may need to consider use of a walking stick, callipers, other orthotic devices and/or orthopaedic footwear, respiratory ventilation, wheelchair or electric scooter. Appropriate advice and referral is important
  • Social and emotional support: support from friends, family or professionals can help people come to terms with changing health or increasing disability. Often people with polio were advised in the past to push their physical limits or to ignore their polio. Many will have developed strategies to manage their condition over the years and will need to adapt again which may take time, support and information
  • Medication considerations: extra care may be needed when prescribing and monitoring side-effects or dosage of certain medications, especially when these can cause or increase muscle or respiratory weakness, fatigue, dizziness and/or drowsiness, depression, insomnia or vasoconstriction. Examples include: anaesthesia or sedation, benzodiazepines, β-blockers, and statins
  • Referral as appropriate: e.g. to a neurologist, orthopaedic consultant, respiratory consultant or specialist respiratory unit, rehabilitation consultant, orthotist, physiotherapist, occupational therapist, speech and language therapist, podiatrist, dietician or pain clinic. Many find self-management or Expert Patient Programmes helpful

Further sources of information

full guidelines available from…
The British Polio Fellowship, Eagle Office Centre, The Runway, South Ruislip, Middlesex, HA4 6SE (Tel –0800 018 0586)
info@britishpolio.org.uk http://www.britishpolio.org.uk

The British Polio Fellowship. Post Polio Syndrome. August 2009
First included: February 1997.