eMedicine Specialties > Physical Medicine and Rehabilitation > Disorders of the Motor Unit

Postpolio Syndrome

Author: Flor M Muñiz, MD, Assistant Professor, Department of Physical Medicine and Rehabilitation, McMaster University, Hamilton, Ontario
Coauthor(s): Gerald J Herbison, MD, Professor, Department of Rehabilitation Medicine, Jefferson Medical College, Thomas Jefferson University
Contributor Information and Disclosures

Updated: May 6, 2009

Introduction

Background

Accepted criteria for diagnosis of postpolio syndrome (PPS) are a prior history of poliomyelitis, a stable period after recovery, a residual deficit of the initial polio, new muscle weakness, and sometimes, new muscle atrophy. Fatigue and muscle pain need not be present to meet the criteria for the syndrome.

Pathophysiology

One possible cause of postpolio syndrome (PPS) is decompensation of a chronic denervation and reinnervation process to the extent that the remaining healthy motor neurons can no longer maintain new sprouts; thus, denervation exceeds reinnervation.1

A second possible mechanism for PPS is motor neuronal loss due to reactivation of a persistent latent virus. In addition to muscle atrophy and denervation, foci of perivascular and interstitial inflammatory cells have been found on 50% of biopsies of patients with PPS. Activated T cells and immunoglobulin M and immunoglobulin G antibodies specific for gangliosides also have been found.

Another possibility is an infection of the polio survivor's motor neurons by an enterovirus that is different from the one responsible for the patient's polio. Others sources hypothesize that PPS is merely the loss of strength due to the usual stresses of aging and weight gain. In patients with PPS, these processes occur in muscles that already are weak, so the consequences are more noticeable compared with those of patients who have not had polio.2

Frequency

United States

The incidence of postpolio syndrome (PPS) in previous acute polio patients ranges from approximately 22-68%. PPS is estimated to occur in 28.5% of persons who had paralytic polio. The current prevalence is approximately 1.6 million cases. Suggestions have been made that 100% of polio survivors, if tracked for a long period, can develop some symptoms of PPS.

Age

The onset of postpolio syndrome is approximately 30 years after the acute polio.

Clinical

History

Symptoms of postpolio syndrome (PPS) usually appear earlier in patients who have very severe residual weakness, individuals who had early bulbar respiratory difficulty in the acute illness, and persons who were older when they contracted acute polio. PPS symptoms tend to occur first in the weaker muscles.

  • Fatigue - In individuals without polio or PPS, the functional consequences of aging and loss of motor units may be unnoticeable until a very advanced age. In the individual with PPS, any further loss of strength may be more readily apparent. In contrast to patients with chronic fatigue syndrome, postpolio fatigue is prominent in the early hours of the afternoon and decreases after brief periods of rest. PPS-related fatigue usually does not prevent patients from working.
    • Central
      • Pathogenesis can include chronic pain, type A personality, depression, dysfunctional reticular-activated system, sleep disorders, and respiratory dysfunction.
      • PPS produces somnolence and difficulty in concentrating and remembering.
    • Peripheral
      • Pathogenesis may be metabolic exhaustion of the enlarged motor units, neuromuscular junction transmission defects, scarring within the motor neurons, or loss of motor units due to aging.
      • PPS produces decreased muscular endurance and increased muscular fatigability.
  • Weakness
    • A number of functional etiologies for weakness have been hypothesized, including disuse, overuse, and chronic weakness, as well as weight gain.
    • Asymmetrical and scattered weakness may be present.
    • Some authors have found evidence that previously unaffected muscles later become weak; in these cases, they discovered that the patient was unaware or had not been told that the particular muscle had been affected during the acute episode.
  • Muscle pain1,3
    • Deep aching pain may be a component of a myofascial pain syndrome or fibromyalgia.
    • This feature is extremely prevalent in PPS. Using a retrospective, cross-sectional survey, Stoelb et al investigated the frequency and most common sites of pain in patients with postpolio syndrome.4 Pain symptoms were reported by 57 (90.5%) of the 63 study participants; pain was reported most often in the shoulders, legs, hips, and lower back, with the most intense pain occurring in the legs, knees, wrists, head, and lower back.
  • Gait disturbance - Difficulty with gait is caused by progressive weakness, pain, osteoarthritis, or joint instability; it is common in patients who previously used assistive devices but later discarded them.
  • Respiratory problems
    • Respiratory disorders are most prevalent in patients with residual respiratory muscle weakness.
    • These changes cause chronic microatelectasis, diminished pulmonary compliance, increased chest wall tightness, chronic alveolar hypoventilation, decreased cough and expiratory flow, and decreased clearing of secretions.
    • The new respiratory difficulties are not only related to new respiratory muscle weakness but also to scoliosis, pulmonary emphysema, cardiovascular insufficiency, or poor posture.
    • A central component also may occur because acute bulbar polio often affects the medullary structures, including the reticular formation and sleep regulatory system.
  • Swallowing problems
    • These difficulties can occur in patients with bulbar and nonbulbar postpolio.
    • Subclinical asymmetrical weakness in the pharyngeal constrictor muscles is almost always present in all postpolio muscular atrophy patients, including those who do not complain of new swallowing difficulties.
  • Autonomic dysfunction - The cause is unclear; the peripheral component could include muscular atrophy and, therefore, diminished heat production.
  • Sleep apnea
    • This disorder is not uncommon in patients left with residual bulbar dysfunction or severe respiratory compromise.
  • Sleep apnea appears to be due to a combination of the following:
    • Central apnea, due to a residual dysfunction of the surviving bulbar reticular neurons
    • Obstructive apnea, due to pharyngeal weakness and increased musculoskeletal deformities from scoliosis or emphysema
    • Postpolio muscular atrophy, resulting in diminished muscle strength of the respiratory, intercostal, and abdominal muscle groups
  • Flat-back syndrome
    • Another possible symptom in some patients with PPS is the flat-back syndrome, which consists of the inability to stand erect because of forward flexion of the trunk and pain in the low back and legs.
    • The flat-back syndrome typically occurs in patients with diminished lumbar lordosis as a result of instrumentation of the spine for scoliosis, vertebral fracture, or degenerative joint disease.
    • The trunk extensor musculature plays an essential role in maintaining upright posture, and it may be that PPS-related weakness in this musculature represents a major contributing factor to the flat-back syndrome in these patients.

Physical

In persons with postpolio syndrome, progressive weakness and atrophy may be observed in muscles that were affected initially by the poliovirus or in muscles that were spared clinically, which tends to happen in an asymmetrical distribution. Fasciculations sometimes can be observed in atrophic muscles, as a result of the lower motor neuron injury.

More on Postpolio Syndrome

Overview: Postpolio Syndrome
Differential Diagnoses & Workup: Postpolio Syndrome
Treatment & Medication: Postpolio Syndrome
Follow-up: Postpolio Syndrome
References
Further Reading
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References

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  2. Bouza C, Munoz A, Amate JM. Postpolio syndrome: a challenge to the health-care system. Health Policy. Jan 2005;71(1):97-106. [Medline].

  3. Fordyce CB, Gagne D, Jalili F, et al. Elevated serum inflammatory markers in post-poliomyelitis syndrome. J Neurol Sci. Aug 15 2008;271(1-2):80-6. [Medline].

  4. Stoelb BL, Carter GT, Abresch RT, et al. Pain in persons with postpolio syndrome: frequency, intensity, and impact. Arch Phys Med Rehabil. Oct 2008;89(10):1933-40. [Medline].

  5. Correa JC, Rocco CC, de Andrade DV, et al. Electromyographic and neuromuscular analysis in patients with post-polio syndrome. Electromyogr Clin Neurophysiol. Nov-Dec 2008;48(8):329-33. [Medline].

  6. Skough K, Krossen C, Heiwe S, et al. Effects of resistance training in combination with coenzyme Q10 supplementation in patients with post-polio: a pilot study. J Rehabil Med. Oct 2008;40(9):773-5. [Medline].

  7. Gonzalez H, Sunnerhagen KS, Sjoberg I, et al. Intravenous immunoglobulin for post-polio syndrome: a randomised controlled trial. Lancet Neurol. Jun 2006;5(6):493-500. [Medline].

  8. On AY, Oncu J, Uludag B, et al. Effects of lamotrigine on the symptoms and life qualities of patients with post polio syndrome: a randomized, controlled study. NeuroRehabilitation. 2005;20(4):245-51. [Medline].

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  14. Dalakas MC. The post-polio syndrome as an evolved clinical entity. Definition and clinical description. Ann N Y Acad Sci. May 25 1995;753:68-80. [Medline].

  15. Diseases of the motor unit. In: DeLisa J, Gans B, eds. Rehabilitation Medicine: Principles and Practice. 3rd ed. Philadelphia, Pa: Lippincott-Raven; 1998:1554-6.

  16. Dinsmore S, Dambrosia J, Dalakas MC. A double-blind, placebo-controlled trial of high-dose prednisone for the treatment of post-poliomyelitis syndrome. Ann N Y Acad Sci. May 25 1995;753:303-13. [Medline].

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  25. Ring D, Vaccaro AR, Scuderi G. An association between the flat back and postpolio syndromes: a report of three cases. Arch Phys Med Rehabil. Mar 1997;78(3):324-6. [Medline].

  26. Semino-Mora C, Dalakas MC. Rimmed vacuoles with beta-amyloid and ubiquitinated filamentous deposits in the muscles of patients with long-standing denervation (postpoliomyelitis muscular atrophy): similarities with inclusion body myositis. Hum Pathol. Oct 1998;29(10):1128-33. [Medline].

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  31. Stein DP, Dambrosia JM, Dalakas MC. A double-blind, placebo-controlled trial of amantadine for the treatment of fatigue in patients with the post-polio syndrome. Ann N Y Acad Sci. May 25 1995;753:296-302. [Medline].

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Further Reading

Clinical guidelines:
EFNS guideline on diagnosis and management of post-polio syndrome. Report of an EFNS task force.
European Federation of Neurological Societies - Medical Specialty Society.  2006 Aug.  7 pages.  NGC:005488

Clinical trials:
Study of Mental Fatigue in Polio Survivors

Related eMedicine topics:
 Acute Poliomyelitis
Breathing-Related Sleep Disorder
Central Sleep Apnea
Enteroviral Infections
Enteroviruses
Focal Muscular Atrophies
Obstructive Sleep Apnea-Hypopnea Syndrome
Poliomyelitis [Orthopedic Surgery]
Poliomyelitis [Pediatrics: General Medicine]
Swallowing Disorders

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Keywords

postpolio syndrome, polio, poliomyelitis, muscle atrophy, post polio, post polio syndrome, sleep apnea, neuromuscular junction, enterovirus, polio virus, poliovirus, denervation, post-polio syndrome, polio complications

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Contributor Information and Disclosures

Author

Flor M Muñiz, MD, Assistant Professor, Department of Physical Medicine and Rehabilitation, McMaster University, Hamilton, Ontario
Flor M Muñiz, MD is a member of the following medical societies: American Medical Association, Canadian Association of Physical Medicine and Rehabilitation, and Canadian Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Gerald J Herbison, MD, Professor, Department of Rehabilitation Medicine, Jefferson Medical College, Thomas Jefferson University
Disclosure: Nothing to disclose.

Medical Editor

Martin K Childers, DO, PhD, Associate Professor, Department of Neurology, Wake Forest University Health Services
Martin K Childers, DO, PhD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Congress of Rehabilitation Medicine, American Osteopathic Association, Christian Medical & Dental Society, and Federation of American Societies for Experimental Biology
Disclosure: Allergan pharma Consulting fee Consulting

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kat Kolaski, MD, Assistant Professor, Departments of Orthopedic Surgery and Pediatrics, Wake Forest University School of Medicine
Kat Kolaski, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine and American Academy of Physical Medicine and Rehabilitation
Disclosure: Nothing to disclose.

CME Editor

Kelly L Allen, MD, Regional Medical Director, IMX-Medical Management Services
Disclosure: Nothing to disclose.

Chief Editor

Denise I Campagnolo, MD, MS, Director of Multiple Sclerosis Clinical Research and Staff Physiatrist, Barrow Neurology Clinics, St Joseph's Hospital and Medical Center; Investigator for Barrow Neurology Clinics; Director, NARCOMS Project for Consortium of MS Centers
Denise I Campagnolo, MD, MS is a member of the following medical societies: Alpha Omega Alpha, American Association of Neuromuscular and Electrodiagnostic Medicine, American Paraplegia Society, Association of Academic Physiatrists, and Consortium of Multiple Sclerosis Centers
Disclosure: Teva Neuroscience Honoraria Speaking and teaching; Serono-Pfizer Honoraria Speaking and teaching

 
 
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