Dr Orlapresentation8


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Dr Orlapresentation8

  1. 1. Poliomyelitis and Post Polio Syndrome Orla Hardiman Beaumont Hospital Dublin
  2. 2. Poliovirus
  3. 3. Poliomyelitis <ul><li>Disease of semi-developed societies </li></ul><ul><li>Occurs in epidemics </li></ul><ul><li>First described in Egypt, major cause of morbidity and mortality until 1960s </li></ul><ul><li>Large epidemics in 1940s and 1950s in developed world, including Ireland </li></ul>
  4. 4. POLIOMYELITIS <ul><li>“ Picornavirus” </li></ul><ul><li>3 types: Poliovirus 1,2,3 </li></ul><ul><li>Ingested, spread by faeco-oral route: Commoner in areas of poor sanitation </li></ul><ul><li>Infants protected by maternal antibodies </li></ul>
  5. 5. Poliomyelitis:Epidemiology <ul><li>“ Silent circulation” Many hundreds may be infected prior to the development of a single case of paralysis </li></ul><ul><li>WHO considers a single confirmed case of polio in an area of low occurrence an epidemic </li></ul>
  6. 6. Epidemiology of Polio in US
  8. 8. Clinical Pattern of Polio
  10. 10. Poliomyelitis:Clinical Features <ul><li>In 1% of cases virus invades CNS: </li></ul><ul><li>Multiples and destroys anterior horn cells. </li></ul><ul><li>In severe cases, poliovirus may attacks motor neurones in brainstem, leading to difficulty in swallowing, speaking and breathing </li></ul>
  11. 11. Poliomyelitis: Risk Factors <ul><li>Immune deficiency </li></ul><ul><li>Pregnancy </li></ul><ul><li>Removal of tonsils </li></ul><ul><li>Intramuscular injections </li></ul><ul><li>Strenuous exercise </li></ul><ul><li>Injury </li></ul>
  12. 12. Measures to Prevent Infection <ul><li>Risk factor identification </li></ul><ul><li>Quarantine </li></ul><ul><li>Hygiene </li></ul><ul><li>Vaccination: “Herd” immunity </li></ul><ul><li>Eradication </li></ul>
  13. 13. Pointers for Parents: (USA 1951)
  14. 14. Poliomyelitis: Treatment <ul><li>No anti-viral agent has yet been developed </li></ul><ul><li>“ Treatment “ is symptomatic </li></ul><ul><li>Supportive care in acute phase, including ventilation if necessary </li></ul><ul><li>Negative pressure ventilators (“iron lung”) used in past </li></ul>
  15. 15. Poliomyelitis: Treatment <ul><li>Intensive physiotherapy </li></ul><ul><li>(Sister Elizabeth Kenney’s method: Hot packs and passive stretching) </li></ul><ul><li>Orthotics </li></ul>
  16. 16. Poliovirus: Eradication <ul><li>Limit infection and dissemination </li></ul><ul><ul><li>Improve general hygiene: Clean water supply </li></ul></ul><ul><li>Polio Vaccines </li></ul><ul><ul><li>Killed virus injected (Salk vaccine: 1955) </li></ul></ul><ul><ul><li>Live attenuated virus (Sabin vaccine 1961) </li></ul></ul>
  17. 17. Inactivated Vaccine <ul><li>Immunity to Poliovirus 1,2,3 </li></ul><ul><li>Safe, effective </li></ul><ul><li>Injection </li></ul><ul><li>No gastrointestinal immunity: Risks of continued circulation of virus in endemic areas </li></ul><ul><li>Expensive </li></ul>Jonas Salk
  18. 18. Live Vaccine <ul><ul><li>Live attenuated oral vaccine (Sabin, 1961): </li></ul></ul><ul><ul><li>Risks of viral mutation, leading to potential regain of virulence: </li></ul></ul><ul><ul><li>Excretion of live virus thru’ faeces </li></ul></ul><ul><ul><li>Live vaccine cheaper, and suitable for mass vaccination programmes </li></ul></ul>
  19. 19. Poliomyelitis in USA Since Vaccinations
  20. 20. Poliomyelitis:Current Status <ul><li>Eradicated from developed world in 1960s </li></ul><ul><li>Remains endemic in 7 countries </li></ul><ul><li>Eradication plan by WHO by year 2000: not yet achieved, but progress is being made </li></ul><ul><li>Methodology more difficult that for smallpox </li></ul>
  21. 21. Polio Eradication: Status in 1988
  22. 22. Polio Eradication: Status in 1998
  23. 24. Polio Revisited <ul><li>5,000 (approx) survivors in Ireland </li></ul><ul><li>Varying degrees of disability </li></ul><ul><li>New health problems associated </li></ul><ul><li>with poliomyelitis infection </li></ul>
  24. 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.
  25. 26. Details Collated <ul><li>Age </li></ul><ul><li>Gender </li></ul><ul><li>Occupation </li></ul><ul><li>Age of onset, </li></ul><ul><li>symptoms at onset </li></ul><ul><li>weakness at onset </li></ul><ul><li>residual weakness </li></ul><ul><li>initial rehabilitation </li></ul><ul><li>Use of callipers/ mobility aids at initial diagnosis </li></ul><ul><li>Surgery </li></ul><ul><li>Current status </li></ul><ul><li>New onset of symptoms </li></ul><ul><li>Concomitant disease </li></ul>
  26. 27. Results <ul><li>9 Misdiagnoses </li></ul><ul><ul><li>Transverse myelitis </li></ul></ul><ul><ul><li>Mononeuropathy </li></ul></ul><ul><ul><li>Cerebral palsy </li></ul></ul><ul><ul><li>Spina bifida </li></ul></ul><ul><ul><li>AVM </li></ul></ul><ul><ul><li>55% affected before 5 years of age </li></ul></ul><ul><li>77% cannot recall symptoms </li></ul><ul><li>15% required respiratory support </li></ul><ul><li>6 vaccine related. </li></ul>
  27. 29. New Symptoms <ul><li>limb weakness (n=38) </li></ul><ul><li>fatigue (n=40) </li></ul><ul><li>increased cold sensitivity (n=4) </li></ul><ul><li>joint pain (n=48) </li></ul><ul><li>low back pain (n=27) </li></ul><ul><li>falls (n=26) </li></ul><ul><li>reduced exercise tolerance (n=31) </li></ul><ul><li>dysphagia (n=4) </li></ul><ul><li>respiratory symptoms (n=5) </li></ul><ul><li>documented muscle weakness and wasting with new disability (n=18). </li></ul>
  28. 31. Natural History of Polio (Halstead)
  29. 32. Criteria For Diagnosis of Post Polio Syndrome <ul><li>A prior episode of paralytic poliomyelitis </li></ul><ul><li>EMG evidence of longstanding denervation </li></ul><ul><li>A period of neurologic recovery and functional stability preceding the onset of new problems (Usually >20 years) </li></ul>
  30. 33. Criteria for Diagnosis of Post Polio Syndrome (cont’d) <ul><li>Gradual or abrupt onset of new non-disuse weakness in previously unaffected or affected muscles </li></ul><ul><li>May be asssociated with fatigue, muscle pain, joint pain, decreased function, etc. </li></ul><ul><li>Exclusion of other conditions that may cause the above features </li></ul>
  31. 34. Pathophysiology <ul><li>Theories : </li></ul><ul><li>Remaining healthy motor neurons can no longer maintain new sprouts </li></ul><ul><li>Decompensation / chronic denervation and reinervation process. </li></ul><ul><li>Denervation exceeds reinervation </li></ul>
  32. 35. Theories (contd.) <ul><li>Motor neuronal loss due to reactivation of a persistant latent virus. </li></ul><ul><li>Infection of the polio survivor’s motor neuron by a different enterovirus </li></ul><ul><li>Loss of strength associated with aging, in already weakened muscles </li></ul>
  33. 36. Possible Causes of Late Complications of Polio
  34. 37. Main Clinical Features of PPS <ul><li>Fatigue (Commonest) </li></ul><ul><li>Weakness </li></ul><ul><li>Muscle pain </li></ul><ul><li>Gait disturbance </li></ul><ul><li>Respiratory problems </li></ul><ul><li>Swallowing problems </li></ul><ul><li>Cold intolerance </li></ul><ul><li>Sleep apnoea </li></ul>
  35. 38. Fatigue <ul><li>Prominent in the early hours of the afternoon </li></ul><ul><li>Decreases with rest </li></ul><ul><li>Pathogenesis:Chronic pain / Muscle pain </li></ul><ul><li>Sleep disorders/ respiratory dysfunction </li></ul><ul><li>Difficulty in remembering/ concentrating </li></ul><ul><li>Decreased muscular endurance / Increased muscular fatigability </li></ul><ul><li>“ Polio wall” </li></ul><ul><li>Generalized or muscular </li></ul>
  36. 39. Weakness <ul><li>Disuse </li></ul><ul><li>Overuse </li></ul><ul><li>Inappropriate use </li></ul><ul><li>Chronic weakness </li></ul><ul><li>Weight gain </li></ul><ul><li>Joint problems </li></ul>
  37. 40. Muscle Pain <ul><li>Extremely prevalent in PPS </li></ul><ul><li>Deep aching pain </li></ul><ul><li>Myofascial pain syndrome / Fibromyalgia </li></ul><ul><li>Small number of patients have muscle tenderness on palpation </li></ul>
  38. 41. Swallowing Problems <ul><li>Can occur in bulbar and non bulbar polio </li></ul><ul><li>Subclinical asymmetrical weakness in the pharyngeal constrictor muscles : almost always present in PPMA (Post polio muscular atrophy) </li></ul><ul><li>Not all are symptomatic </li></ul>
  39. 42. Cold Intolerance <ul><li>Autonomic nervous system dysfunction? </li></ul><ul><li>May relate to sympathetic intermediolateral column damage during acute poliomyelitis </li></ul><ul><li>Peripheral component may include muscular atrophy leading to reduced heat production </li></ul>
  40. 43. Sleep Apnoea <ul><li>Combination of the following: </li></ul><ul><li>Central: residual dysfunction of surviving bulbar reticular neurons </li></ul><ul><li>Obstructive: pharyngeal weakness and increased musculoskeletal deformities from scoliosis or emphysema </li></ul><ul><li>PPMA, diminished muscle strength of respiratory,intercostal & abdominal muscle groups </li></ul>
  41. 44. Risk Factors for Sleep Apnoea <ul><li>Age of onset (More severe disease in adolescents and adults) </li></ul><ul><li>Severity of original paralysis </li></ul><ul><li>Managed with BiPAP </li></ul>
  42. 45. Management of Post Polio Syndrome in Ireland <ul><li>Assessment </li></ul><ul><li>Exclusion of other causes of disability </li></ul><ul><li>Introduction to concept of interdisciplinary team </li></ul><ul><li>Follow-up as necessary </li></ul>
  43. 46. Post Polio Syndrome Multidisciplinary Team <ul><ul><li>Neurologist </li></ul></ul><ul><ul><li>Rehabilitation physician </li></ul></ul><ul><ul><li>Rheumatologist </li></ul></ul><ul><ul><li>Respiratory physician </li></ul></ul><ul><ul><li>Voluntary organization </li></ul></ul><ul><li>Clinical Professional Services: </li></ul><ul><ul><li>Physiotherapy </li></ul></ul><ul><ul><li>Occupational Therapy </li></ul></ul><ul><ul><li>Speech and Language Therapy </li></ul></ul><ul><ul><li>Social Services </li></ul></ul>
  44. 47. Management of Post Polio Syndrome in Ireland <ul><li>Evaluation: </li></ul><ul><ul><li>Neurologic Examination to define nature of new weakness (neurogenic v disuse) </li></ul></ul><ul><ul><li>Neurophysiology </li></ul></ul><ul><ul><li>Pulmonary Function studies, polysomnography if necessary </li></ul></ul><ul><ul><li>Rheumatology /rehabilitation assessment </li></ul></ul><ul><ul><li>Swallowing study: Aspiration risk </li></ul></ul>
  45. 48. Management of Post Polio Syndrome in Ireland <ul><li>Radiography </li></ul><ul><ul><li>Chest (aspiration, Diaphragmatic paresis) </li></ul></ul><ul><ul><li>Joints (arthritis) </li></ul></ul>
  46. 49. Management of Post Polio Syndrome in Ireland <ul><li>Specialised Orthotics </li></ul><ul><li>Community-based Services </li></ul><ul><li>Access to free medical care and disability-based tax exemptions </li></ul>
  47. 50. Measuring Progression <ul><li>6 monthly quantitative muscle assessment </li></ul><ul><li>Measurement of strength in individual muscles </li></ul><ul><li>Identification of rate of progression in PPMA </li></ul>
  50. 53. Research <ul><li>Maximum Voluntary Isometric Contraction: Serial testing at 6 month intervals </li></ul><ul><li>Detailed electromyography </li></ul><ul><li>Fatigue measurement & correlation with muscle strength </li></ul><ul><li>Tests for Diabetes Mellitus </li></ul>
  51. 54. ELECTROMYOGRAPHY <ul><li>Abnormal in all people who had polio </li></ul><ul><li>Distinctive pattern in people with PPS </li></ul>
  52. 55. Treatment /Management <ul><li>Recognition </li></ul><ul><li>Symptomatic and supportive </li></ul><ul><ul><li>Occupational therapy: orthotics etc </li></ul></ul><ul><li>Fatigue /sleepiness </li></ul><ul><ul><li>Look for features of sleep apnoea </li></ul></ul><ul><ul><li>energy conservation </li></ul></ul>
  53. 56. CONCLUSIONS <ul><li>Polio may have been over diagnosed in the past </li></ul><ul><li>PPS is under-recognised </li></ul><ul><li>Specialist clinic is beneficial </li></ul><ul><li>Management is multidisciplinary </li></ul><ul><li>Many research questions remain </li></ul>