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Dr Orlapresentation8 Dr Orlapresentation8 Presentation Transcript

  • Poliomyelitis and Post Polio Syndrome Orla Hardiman Beaumont Hospital Dublin
  • Poliovirus
  • 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
  • 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
  • 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
  • Epidemiology of Polio in US
  • LIFE CYCLE OF POLIO VIRUS
  • Clinical Pattern of Polio
  • POLIO ATTACKS MOTOR NEURONES
  • 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
  • Poliomyelitis: Risk Factors
    • Immune deficiency
    • Pregnancy
    • Removal of tonsils
    • Intramuscular injections
    • Strenuous exercise
    • Injury
  • Measures to Prevent Infection
    • Risk factor identification
    • Quarantine
    • Hygiene
    • Vaccination: “Herd” immunity
    • Eradication
  • Pointers for Parents: (USA 1951)
  • 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
  • Poliomyelitis: Treatment
    • Intensive physiotherapy
    • (Sister Elizabeth Kenney’s method: Hot packs and passive stretching)
    • Orthotics
  • 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)
  • 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
  • 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
  • Poliomyelitis in USA Since Vaccinations
  • 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
  • Polio Eradication: Status in 1988
  • Polio Eradication: Status in 1998
  •  
  • Polio Revisited
    • 5,000 (approx) survivors in Ireland
    • Varying degrees of disability
    • New health problems associated
    • with poliomyelitis infection
  • 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.
  • 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
  • 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.
  •  
  • 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).
  •  
  • Natural History of Polio (Halstead)
  • 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)
  • 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
  • Pathophysiology
    • Theories :
    • Remaining healthy motor neurons can no longer maintain new sprouts
    • Decompensation / chronic denervation and reinervation process.
    • Denervation exceeds reinervation
  • 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
  • Possible Causes of Late Complications of Polio
  • Main Clinical Features of PPS
    • Fatigue (Commonest)
    • Weakness
    • Muscle pain
    • Gait disturbance
    • Respiratory problems
    • Swallowing problems
    • Cold intolerance
    • Sleep apnoea
  • 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
  • Weakness
    • Disuse
    • Overuse
    • Inappropriate use
    • Chronic weakness
    • Weight gain
    • Joint problems
  • Muscle Pain
    • Extremely prevalent in PPS
    • Deep aching pain
    • Myofascial pain syndrome / Fibromyalgia
    • Small number of patients have muscle tenderness on palpation
  • 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
  • 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
  • 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
  • Risk Factors for Sleep Apnoea
    • Age of onset (More severe disease in adolescents and adults)
    • Severity of original paralysis
    • Managed with BiPAP
  • Management of Post Polio Syndrome in Ireland
    • Assessment
    • Exclusion of other causes of disability
    • Introduction to concept of interdisciplinary team
    • Follow-up as necessary
  • 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
  • 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
  • Management of Post Polio Syndrome in Ireland
    • Radiography
      • Chest (aspiration, Diaphragmatic paresis)
      • Joints (arthritis)
  • Management of Post Polio Syndrome in Ireland
    • Specialised Orthotics
    • Community-based Services
    • Access to free medical care and disability-based tax exemptions
  • Measuring Progression
    • 6 monthly quantitative muscle assessment
    • Measurement of strength in individual muscles
    • Identification of rate of progression in PPMA
  • SERIAL QMAs IN 16 MUSCLES
  • CHANGES IN STRENGTH OVER TIME USING QMA
  • Research
    • Maximum Voluntary Isometric Contraction: Serial testing at 6 month intervals
    • Detailed electromyography
    • Fatigue measurement & correlation with muscle strength
    • Tests for Diabetes Mellitus
  • ELECTROMYOGRAPHY
    • Abnormal in all people who had polio
    • Distinctive pattern in people with PPS
  • Treatment /Management
    • Recognition
    • Symptomatic and supportive
      • Occupational therapy: orthotics etc
    • Fatigue /sleepiness
      • Look for features of sleep apnoea
      • energy conservation
  • 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