Post-Polio Syndrome

Introduction[edit | edit source]

Post-polio syndrome (PPS) is a condition that affects Polio survivors after years of recovery from initial Poliovirus infection. PPS is a result of the deterioration of motor neurons which leads to loss of muscle strength and dysfunction[1]. Polio cases have decreased by over 99% since 1988, from an estimated 350,000 cases to 175 reported cases in 2019 and only 6 cases were reported in 2021[2].  Poliomyelitis and Post-polio syndrome are both caused by poliovirus but Poliomyelitis is contagious whereas PPS is not. Only a polio survivor is at risk of developing PPS however not all polio survivors develop PPS[3].

Polio sequelle.jpg

Pathological Process[edit | edit source]

Acute poliomyelitis affects the limbs and respiratory musculature and causes bulbar manifestations. The degeneration of the anterior horn and its apoptosis is considered as a hallmark of paralytic poliomyelitis. Following this acute phase, axonal sprouting occurs reinnervating the affected muscles. The motor units become abnormally enlarged making them metabolically unsustainable which can take decades to happen from the poliovirus infection to the occurrence of PPS[4]. Reinnervated motor units' progressive degeneration, their increased metabolic demand leading to neuromuscular junction dysfunction, the persistence of the poliovirus in neural tissue, and induction of autoimmunity, which results in the destruction of neural structures are all evidenced by electromyography (EMG) findings, muscle histology and muscle fiber-type grouping[5].

Clinical Presentation[edit | edit source]

Understanding the symptoms of PPS is important for correct diagnosis and management. Symptoms can range from fatigue and. muscle weakness to more severe issues that can affect mobility and breathing.

  1. Fatigue is the most common symptom of post-polio syndrome and it occurs in about 80% of patients.[6][7]It can take many forms, including:
    • muscle fatigue – where your muscles feel very tired and heavy, particularly after physical activity
    • general fatigue – where you feel an overwhelming sense of physical exhaustion as if you've not slept for days
    • mental fatigue – where you find it increasingly difficult to concentrate, have problems remembering things and make mistakes you wouldn't usually make
  2. Muscle weakness: An increase in muscle weakness is another common symptom of post-polio syndrome.Muscle weakness is where you are finding it difficult to use the affected muscles, whether you feel tired or not. Weakness can occur in the muscles that were previously affected by the polio infection.[8] It can also affect those that weren't previously substantially affected.[9] There may also be associated shrinking of affected muscles, known as atrophy. Increasing muscle weakness is another common symptom of post-polio syndrome. It can be easy to confuse muscle weakness with muscle fatigue, but they are different.
  3. Muscle and joint pain: Muscle and joint pain are also common in post-polio syndrome. Muscle pain is usually felt deep like an ache in the muscles or muscle cramps and spasms. The pain is often worse in the evenings and is similar to arthritis and consists of soreness, stiffness and a reduced range of movement.[1]Muscle and joint pain are also common in post-polio syndrome. Muscle pain is usually felt as a deep ache in the muscles or muscle cramps and spasms. The pain is often worse after you've used the affected muscles. It can be particularly troublesome during the evening after a day's activities.
  4. Weight gain: With all of the above symptoms, most people with post-polio syndrome become less physically active than they used to be. This can often lead to weight gain and in some cases, obesity. Because of the symptoms mentioned above, most people with post-polio syndrome become less physically active than they used to be. This in turn can make any fatigue, muscle weakness, and pain worse.
  5. Mobility difficulties: The combination of fatigue, weakness and pain can also make walking difficult, leading to increasing mobility problems. Often people with post-polio syndrome will need a walking aid, such as crutches or a stick at some stage, and some people may eventually need to use a wheelchair. As well as weight gain, the combination of fatigue, weakness and pain can also make walking difficult, leading to increasing mobility problems. The dependence on assistive technology increases and even requires orthotic bracing.
  6. Breathing difficulties: Some people with post-polio syndrome find breathing can be difficult because the breathing muscles become weaker. This can cause problems such as respiratory insufficiency, shortness of breath during sleep, sleep apnea, which has a prevalence of 65%.[10] There is also an increased risk of chest infections due to the muscle weakness. It's important to get medical advice as soon as possible if you experience symptoms of a possible chest infection, such as coughing up discoloured phlegm or blood, chest pain and wheezing.
  7. Swallowing problems: Weakness in the muscles you use for chewing and swallowing (pharyngeal and laryngeal muscles) may lead to problems swallowing (dysphagia), such as coughing, choking or gagging when swallowing.
  8. Speech Problems: Voice and speech changes many occur, such as hoarseness, low volume or basal sounding voice, particularly after you have been speaking for a while or when your are tired. This is usually due to facial weakness and vocal cord paralysis.[8]
  9. Sensitivity to cold: People of post polio syndrome often suffer from cold intolerance Its prevalence ranges from 29 to 56%. There is inadequate muscle support for vasoconstriction of blood vessels required to reduce the amount of warm blood flow to the skin and to conserve body heat. Treatment such as biofeedback, visualization or relaxation can help to increase warm blood in PPS. [11]

Diagnosis[edit | edit source]

Diagnosis of PPS can be challenging due to the lack of specific tests. Several criteria and tests exist to differentiat PPS from other conditions with similar symptoms, they include:

  • Prior poliomyelitis (paralytic) and evidence of motor neuron loss as confirmed by history of acute paralytic illness , signs of muscle atrophy and residual weakness and denervation on EMG.
  • A period of partial or complete functional recovery after acute paralytic poliomyleitis , followed by an interval(usually 15years or more) of stable NM function.
  • Gradual onset(abrupt in rare case) progressive and persistent new muscle weakness or abnormal muscle fatigability (decreased endurance) with or without generalized ,muscle atrophy , muscle and joint dysfunction or respiratory weakness occurs.
  • Symptoms that persist for atleast a year.
  • Exclusion of alternative NM , medical and orthopedic problems as causes of symptoms.[12]

As the symptoms of post-polio syndrome can be similar to those of several other conditions, such as arthritis, some tests such as X-rays may be needed to rule out any other possible causes of your problems.

To rule out other conditions, or confirm whether you have post-polio syndrome, the following tests may be carried out:

  • Electromyography (EMG) tests – to measure the electrical activity in your muscles and nerves and find out whether they're damaged.
  • Sleep studies – if you're having problems sleeping, such as sleep apnea, or you're feeling unusually tired.
  • Heart rate and function tests.
  • A magnetic resonance imaging (MRI) scan or computer tomography (CT).
  • Lung function tests .
  • Tests to investigate swallowing problems (dysphagia).

It's possible to have post-polio syndrome alongside other conditions, so not every health problem or symptom you experience may be related to the condition.

Management[edit | edit source]

The management of Post-polio syndrome involves a multidisciplinary team from physical therapists, occupational therapist, speech and language pathologists to neurologists and physiatrists. Occupational therapists can address upper extremity problems, especially overuse injuries, as well as recommend adaptive equipment for your home and office. Speech and language pathologists evaluate and treat swallowing and speech problems. The main goals in physical therapy are to improve muscle strength and endurance, prevent falls and improve balance. Muscular strengthening can be indicated for PPS to allow patients to regain some muscle strength. If the subject is in a phase of progressive loss of strength, muscle conditioning is still possible but its intensity must remain moderate and it should be fractioned with periods of rest, it must be reassessed very regularly and stopped if there is pain or a worsening of muscular fatigue[13]. Endurance rehabilitation is also indicated where patients exercise two to three times a week for short periods (20 to 40 minutes). The training was always moderate respecting a threshold of 70% of the theoretical HRmax[14]. In addition to that, aquatic therapy is the treatment of choice for PPS because this form of therapy allows muscular training in a controlled resistance environment and to perform an assisted active autonomous workout on deficient muscles in an antigravity setting, thus creating the proper conditions for relieving musculoskeletal pain[15].

Differential Diagnosis[edit | edit source]

It is important to differentiate PPS from other conditions that may present with similar symptoms, including:[16][17][18][19][20][21]

Summary[edit | edit source]

Post-polio syndrome is a complex condition affecting polio survivors, often manifesting years after the initial recovery. With a range of symptoms that can impact various aspects of daily life, from muscle strength and mobility to breathing and even mental well-being, it presents unique challenges for both diagnosis and management. The condition often necessitates a multidisciplinary approach to healthcare, involving professionals like physical therapists, occupational therapists, and neurologists. While diagnosis can be challenging due to the lack of specific tests, various criteria and assessments can help differentiate it from conditions with similar symptoms. Effective management strategies exist, but they often require ongoing, tailored interventions.

References[edit | edit source]

  1. 1.0 1.1 NHS Post-polio syndrome https://www.nhs.uk/conditions/post-polio-syndrome/ (Accessed 27th September 2020)
  2. Bigouette JP, Wilkinson AL, Tallis G, Burns CC, Wassilak SG, Vertefeuille JF. Progress toward polio eradication—worldwide, January 2019–June 2021. Morbidity and Mortality Weekly Report. 2021 Aug 8;70(34):1129.
  3. Leon-Monzon ME, Dalakas MC. Detection of poliovirus antibodies and poliovirus genome in patients with the post-polio syndrome. Annals of the New York Academy of Sciences. 1995 May 1;753:208-18.
  4. Luciano CA, Sivakumar K, Spector SA, Dalakas MC. Reinnervation in Clinically Unaffected Muscles of Patients with Prior Paralytic Poliomyelitis. Annals of the New York Academy of Sciences. 1995 May 1;753(1):394-401.
  5. Cashman NR, Trojan DA. Correlation of electrophysiology with pathology, pathogenesis, and anticholinesterase therapy in post-polio syndrome. Annals of the New York Academy of Sciences-Paper Edition. 1995 May 25;753:138-50.
  6. Agre JC, Grimby G, Rodriquez AA, Einarsson G, Swiggum ER, Franke TM. A comparison of symptoms between Swedish and American post-polio individuals and assessment of lower limb strength--a four-year cohort study. Scand J Rehabil Med. 1995 Sep;27(3):183-92. PMID: 8602481.
  7. Jubelt B, Drucker J. Post-polio syndrome: an update. Semin Neurol. 1993 Sep;13(3):283-90. doi: 10.1055/s-2008-1041136. PMID: 8272600.
  8. 8.0 8.1 Simionescu L, Modlin JF. Poliomyelitis and post-polio syndrome. UpToDate. Accessed September. 2022;1.
  9. Dalakas M, Illa I. Post-polio syndrome: concepts in clinical diagnosis, pathogenesis, and etiology. Adv Neurol. 1991;56:495-511. PMID: 1853779.
  10. Léotard A, Lévy J, Hartley S, Pages A, Genet F, Lofaso F, Prigent H, Quera-Salva MA. Sleep disorders in aging polio survivors: A systematic review. Ann Phys Rehabil Med. 2020 Nov;63(6):543-553. doi: 10.1016/j.rehab.2019.10.007. Epub 2019 Nov 30. PMID: 31794858.
  11. Oluwasanmi OJ, Mckenzie DA, Adewole IO, Aluka CO, Iyasse J, Olunu E, Fakoya AO. Postpolio syndrome: a review of lived experiences of patients. International Journal of Applied and Basic Medical Research. 2019 Jul;9(3):129.
  12. Li Hi Shing S, Chipika RH, Finegan E, Murray D, Hardiman O, Bede P. Post-polio syndrome: more than just a lower motor neuron disease. Frontiers in Neurology. 2019:773.
  13. Halstead LS, Gawne AC, Pham BT. National rehabilitation hospital limb classification for exercise, research, and clinical trials in post-polio patients. Annals of the New York Academy of Sciences-Paper Edition. 1995 May 1;753:343-53.
  14. Kriz JL, Jones DR, Speier JL, Canine JK, Owen RR, Serfass RC. Cardiorespiratory responses to upper extremity aerobic training by postpolio subjects. Archives of physical medicine and rehabilitation. 1992 Jan 1;73(1):49-54.
  15. Willén C, Scherman MH. Group training in a pool causes ripples on the water: experiences by persons with late effects of polio. Journal of rehabilitation medicine. 2002 Jul 1;34(4):191-7.
  16. Lo JK, Robinson LR. Postpolio syndrome and the late effects of poliomyelitis. Part 1. pathogenesis, biomechanical considerations, diagnosis, and investigations. Muscle Nerve. 2018 Dec;58(6):751-759. doi: 10.1002/mus.26168. Epub 2018 Aug 22. PMID: 29752819.
  17. Trojan DA, Cashman NR. Fibromyalgia is common in a postpoliomyelitis clinic. Arch Neurol. 1995 Jun;52(6):620-4. doi: 10.1001/archneur.1995.00540300094019. PMID: 7763212.
  18. Verma A, Bradley WG. Atypical motor neuron disease and related motor syndromes. Semin Neurol. 2001 Jun;21(2):177-87. doi: 10.1055/s-2001-15263. PMID: 11442326.
  19. Oluwasanmi OJ, Mckenzie DA, Adewole IO, Aluka CO, Iyasse J, Olunu E, Fakoya AO. Postpolio Syndrome: A Review of Lived Experiences of Patients. Int J Appl Basic Med Res. 2019 Jul-Sep;9(3):129-134. doi: 10.4103/ijabmr.IJABMR_333_18. PMID: 31392174; PMCID: PMC6652271.
  20. Verma R, Lalla R, Sahu R. Hypothyroid myopathy mimicking postpolio syndrome. BMJ Case Rep. 2012 Aug 24;2012:bcr2012006554. doi: 10.1136/bcr-2012-006554. PMID: 22922922; PMCID: PMC4544315.
  21. LaBan MM, Sanitate SS, Taylor RS. Spinal stenosis presenting as "the postpolio syndrome". Review of four cases. Am J Phys Med Rehabil. 1993 Dec;72(6):390-4. PMID: 8260134.