Poliomyelitis, or polio, is a viral disease that was a major cause of disability until widespread vaccination in the 1960s. Some polio survivors later develop Post-Polio Syndrome, characterized by new muscle weakness and fatigue decades after the initial infection. Management of Post-Polio Syndrome is multidisciplinary and focuses on symptom management through rehabilitation, orthotics, energy conservation, and treatment of comorbidities like sleep apnea. While polio has been eradicated in most countries, Post-Polio Syndrome remains an issue for aging polio survivors and further research is still needed.

1. Poliomyelitis and Post Polio Syndrome Orla Hardiman Beaumont Hospital Dublin

2. Poliovirus

3. Poliomyelitis Disease of semi-developed societies Occurs in epidemics First described in Egypt, major cause of morbidity and mortality until 1960s Large epidemics in 1940s and 1950s in developed world, including Ireland

4. POLIOMYELITIS “ Picornavirus” 3 types: Poliovirus 1,2,3 Ingested, spread by faeco-oral route: Commoner in areas of poor sanitation Infants protected by maternal antibodies

5. Poliomyelitis:Epidemiology “ Silent circulation” Many hundreds may be infected prior to the development of a single case of paralysis WHO considers a single confirmed case of polio in an area of low occurrence an epidemic

6. Epidemiology of Polio in US

7. LIFE CYCLE OF POLIO VIRUS

8. Clinical Pattern of Polio

9. POLIO ATTACKS MOTOR NEURONES

10. Poliomyelitis:Clinical Features In 1% of cases virus invades CNS: Multiples and destroys anterior horn cells. In severe cases, poliovirus may attacks motor neurones in brainstem, leading to difficulty in swallowing, speaking and breathing

11. Poliomyelitis: Risk Factors Immune deficiency Pregnancy Removal of tonsils Intramuscular injections Strenuous exercise Injury

12. Measures to Prevent Infection Risk factor identification Quarantine Hygiene Vaccination: “Herd” immunity Eradication

13. Pointers for Parents: (USA 1951)

14. Poliomyelitis: Treatment No anti-viral agent has yet been developed “ Treatment “ is symptomatic Supportive care in acute phase, including ventilation if necessary Negative pressure ventilators (“iron lung”) used in past

15. Poliomyelitis: Treatment Intensive physiotherapy (Sister Elizabeth Kenney’s method: Hot packs and passive stretching) Orthotics

16. Poliovirus: Eradication Limit infection and dissemination Improve general hygiene: Clean water supply Polio Vaccines Killed virus injected (Salk vaccine: 1955) Live attenuated virus (Sabin vaccine 1961)

17. Inactivated Vaccine Immunity to Poliovirus 1,2,3 Safe, effective Injection No gastrointestinal immunity: Risks of continued circulation of virus in endemic areas Expensive Jonas Salk

18. Live Vaccine Live attenuated oral vaccine (Sabin, 1961): Risks of viral mutation, leading to potential regain of virulence: Excretion of live virus thru’ faeces Live vaccine cheaper, and suitable for mass vaccination programmes

19. Poliomyelitis in USA Since Vaccinations

20. Poliomyelitis:Current Status Eradicated from developed world in 1960s Remains endemic in 7 countries Eradication plan by WHO by year 2000: not yet achieved, but progress is being made Methodology more difficult that for smallpox

21. Polio Eradication: Status in 1988

22. Polio Eradication: Status in 1998

24. Polio Revisited 5,000 (approx) survivors in Ireland Varying degrees of disability New health problems associated with poliomyelitis infection

25. THE POST POLIO SYNDROME: EXPERIENCE FROM A TERTIARY NEUROLOGY REFERRAL CENTRE IN IRELAND Dr. Grainne Gorman,Catherine Lynch R.N.,Dr. Orla Hardiman Department of Neurology, Beaumont Hospital.

26. Details Collated Age Gender Occupation Age of onset, symptoms at onset weakness at onset residual weakness initial rehabilitation Use of callipers/ mobility aids at initial diagnosis Surgery Current status New onset of symptoms Concomitant disease

27. Results 9 Misdiagnoses Transverse myelitis Mononeuropathy Cerebral palsy Spina bifida AVM 55% affected before 5 years of age 77% cannot recall symptoms 15% required respiratory support 6 vaccine related.

29. New Symptoms limb weakness (n=38) fatigue (n=40) increased cold sensitivity (n=4) joint pain (n=48) low back pain (n=27) falls (n=26) reduced exercise tolerance (n=31) dysphagia (n=4) respiratory symptoms (n=5) documented muscle weakness and wasting with new disability (n=18).

31. Natural History of Polio (Halstead)

32. Criteria For Diagnosis of Post Polio Syndrome A prior episode of paralytic poliomyelitis EMG evidence of longstanding denervation A period of neurologic recovery and functional stability preceding the onset of new problems (Usually >20 years)

33. Criteria for Diagnosis of Post Polio Syndrome (cont’d) Gradual or abrupt onset of new non-disuse weakness in previously unaffected or affected muscles May be asssociated with fatigue, muscle pain, joint pain, decreased function, etc. Exclusion of other conditions that may cause the above features

34. Pathophysiology Theories : Remaining healthy motor neurons can no longer maintain new sprouts Decompensation / chronic denervation and reinervation process. Denervation exceeds reinervation

35. Theories (contd.) Motor neuronal loss due to reactivation of a persistant latent virus. Infection of the polio survivor’s motor neuron by a different enterovirus Loss of strength associated with aging, in already weakened muscles

36. Possible Causes of Late Complications of Polio

37. Main Clinical Features of PPS Fatigue (Commonest) Weakness Muscle pain Gait disturbance Respiratory problems Swallowing problems Cold intolerance Sleep apnoea

38. Fatigue Prominent in the early hours of the afternoon Decreases with rest Pathogenesis:Chronic pain / Muscle pain Sleep disorders/ respiratory dysfunction Difficulty in remembering/ concentrating Decreased muscular endurance / Increased muscular fatigability “ Polio wall” Generalized or muscular

39. Weakness Disuse Overuse Inappropriate use Chronic weakness Weight gain Joint problems

40. Muscle Pain Extremely prevalent in PPS Deep aching pain Myofascial pain syndrome / Fibromyalgia Small number of patients have muscle tenderness on palpation

41. Swallowing Problems Can occur in bulbar and non bulbar polio Subclinical asymmetrical weakness in the pharyngeal constrictor muscles : almost always present in PPMA (Post polio muscular atrophy) Not all are symptomatic

42. Cold Intolerance Autonomic nervous system dysfunction? May relate to sympathetic intermediolateral column damage during acute poliomyelitis Peripheral component may include muscular atrophy leading to reduced heat production

43. Sleep Apnoea Combination of the following: Central: residual dysfunction of surviving bulbar reticular neurons Obstructive: pharyngeal weakness and increased musculoskeletal deformities from scoliosis or emphysema PPMA, diminished muscle strength of respiratory,intercostal & abdominal muscle groups

44. Risk Factors for Sleep Apnoea Age of onset (More severe disease in adolescents and adults) Severity of original paralysis Managed with BiPAP

45. Management of Post Polio Syndrome in Ireland Assessment Exclusion of other causes of disability Introduction to concept of interdisciplinary team Follow-up as necessary

46. Post Polio Syndrome Multidisciplinary Team Neurologist Rehabilitation physician Rheumatologist Respiratory physician Voluntary organization Clinical Professional Services: Physiotherapy Occupational Therapy Speech and Language Therapy Social Services

47. Management of Post Polio Syndrome in Ireland Evaluation: Neurologic Examination to define nature of new weakness (neurogenic v disuse) Neurophysiology Pulmonary Function studies, polysomnography if necessary Rheumatology /rehabilitation assessment Swallowing study: Aspiration risk

48. Management of Post Polio Syndrome in Ireland Radiography Chest (aspiration, Diaphragmatic paresis) Joints (arthritis)

49. Management of Post Polio Syndrome in Ireland Specialised Orthotics Community-based Services Access to free medical care and disability-based tax exemptions

50. Measuring Progression 6 monthly quantitative muscle assessment Measurement of strength in individual muscles Identification of rate of progression in PPMA

51. SERIAL QMAs IN 16 MUSCLES

52. CHANGES IN STRENGTH OVER TIME USING QMA

53. Research Maximum Voluntary Isometric Contraction: Serial testing at 6 month intervals Detailed electromyography Fatigue measurement & correlation with muscle strength Tests for Diabetes Mellitus

54. ELECTROMYOGRAPHY Abnormal in all people who had polio Distinctive pattern in people with PPS

55. Treatment /Management Recognition Symptomatic and supportive Occupational therapy: orthotics etc Fatigue /sleepiness Look for features of sleep apnoea energy conservation

56. CONCLUSIONS Polio may have been over diagnosed in the past PPS is under-recognised Specialist clinic is beneficial Management is multidisciplinary Many research questions remain