Successfully reported this slideshow.
We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. You can change your ad preferences anytime.

Dr Orlapresentation8


Published on

Published in: Health & Medicine

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>