Diagnosis and Medical Management of Post-Polio Syndrome

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Diagnosis and Medical Management of Post-Polio Syndrome Powered By Docstoc
					Diagnosis and Medical
   Management of
Post-Polio Syndrome
     Dr Michael Watt
   Consultant Neurologist
       RVH, Belfast
How easy it is to Forget
   What is PPS?

   Have I got it or have I got something else?

   What can I do about it?
                       History of PPS
   First case described in 1875 (Raymond, 1875)

   Zilkha (1962) described 11 cases occurring 17-43 years post
    acute illness.

   Halstead (1985) – “post polio syndrome”, (PPS), then, re-
    defined it in 1991.

   Dalakas (1995) defined post polio muscular atrophy (PPMA)

   Berg(1996) “Post Polio Muscular dysfunction” (PPMD)

   Howard (1988,2003)) Post-polio functional deterioration
    (PPFD)
                 Halstead’s 1985 Definition
   Confirmed history of polio
   Partial or fairly complete neurological and functional recovery
    after the acute episode.
   Period of at least 15 years with neurological and functional
    stability
   Two or more of the following health problems occurring after
    the stable period:
       Extensive fatigue
       Muscle and or joint pain
       New weakness in muscles previously affected or unaffected
       New muscle atrophy
       Functional loss
       Cold intolerance
   No other medical explanation found
                         Prospective study of New symptoms
      NEW DIFFICULTIES EXPERIENCED BY SUBJECTS WITH PRIOR PARALYTIC
                                 POLIO*
                                                                                                                             Patients
Symptom                                                       No.                           Requiring New Aids                            Changing Activities
Fatigue alone                                                    2                                            1                                            0
Pain alone                                                      7§                                            0                                            1
Pain and fatigue                                                 1                                            1                                            0
Weakness alone                                                   4                                            0                                            0
Weakness and pain                                                9                                            2                                            0
Weakness and fatigue                                             2                                            1                                            0
Weakness, pain and
                                                                 7                                            2                                            1
fatigue
No new symptoms                                                 18                                            0                                            0

* Types of complaints reported by 32 of the 50 subjects with paralytic polio. Twenty-two complained of some new weakness. In seven subjects, the new symptoms necessitated the use
of new aids to daily living and in two different cases, the symptoms had led to lifestyle changes.
§ All seven complained of nonradiating lumbar or cervical pain.
Windebank AJ et al. Late effects of paralytic poliomyelitis in Omsted County, Minnesota. Neurology. 1991; 41:507-507
  MOST COMMON NEW HEALTH AND FUNCTIONAL PROBLEMS OF PATIENTS WITH
         CONFIRMED POLIO EVALUATED IN TWO POST-POLIO CLINICS
                               Texas*               Wisconsin§
                              (N = 132)              (N = 79)
                        N                 %    N                 %
HEALTH PROBLEMS
Fatigue                 117               89   68                86
Muscle pain             93                71   68                86
Joint pain              93                71   61                77
Weakness:

     Affected muscles   91                69   63                80

     Unaffected
                        66                50   42                53
     muscles
Atrophy                 37                28   31                39
FUNCTIONAL
PROBLEMS
Walking                 84                64   --                --
Climbing stairs         80                61   53                67
Dressing                23                17   13                16
             Epidemiology of PPS

   The frequency of PPS ranges between 15%-
    80%, depending which population are studied,
    and which criteria are applied.

   In European populations a prevalence of
    between 46% (Holland) and 60% (Edinburgh,
    Norway, Denmark) is seen in the literature.
What Causes PPS?
Motor Neuron Loss?
             CNS
                                          CNS




Normal:
One nerve/motor muscle unit
                              PPS:
                              multiple motor units /nerve
               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 persistent
    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
                       What Causes PPS?

   Accelerated natural ageing
   Falling nerve to muscle motor unit ratio
   Inflammation and active immune response
   Co-morbidity:
       Orthopaedic problems
       Radiculopathy and entrapment neuropathy
       Respiratory failure
       General medical problems
   PPS is more likely with
       increasing age;
       the more severe the initial weakness was
       The more time that elapses after the attack of polio
        Non-paralytic polio and PPS?

   For non-paralytic polio it is impossible to
    exclude a scaled down version of the same
    processes.
   Such a diagnosis however is presumptive and
    cannot be categorically confirmed.
   When we have further knowledge about the
    specificity and sensitivity of EMG, muscle
    biopsy and immunological tests it should be
    possible to give more definite diagnoses
    Main Clinical Features of PPS

   Fatigue (Commonest)      Respiratory problems
   Weakness                 Swallowing problems
   Muscle pain              Cold intolerance
   Gait disturbance         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
Is it PPS?


Other
neuromuscular
diseases



Nerve   entrapment
Is it PPS?

Spinal cord and
nerve root problems

Scoliosis
       Is it PPS? – Other things to think of
   Other rheumatological disorders: rheumatoid
    arthritis, lupus, Sjorgren’s syndrome or just
    osteoarthritis
   Endocrine disorders: hypothyroidism, adrenal
    failure, rarely pituitary failure
   Orthopaedic problems: shoulder rotator cuff tears
    and impingement syndrome, spondylosis, bursitis,
    metatarsalgia.
   Breathing disorders: restrictive problems with
    scoliosis, obstructive sleep apnoea
   General medical problems: heart failure, diabetes
             How is it Investigated?

   MRI scans
   Blood tests
   EMG and nerve conduction studies
   X-rays
   Overnight oximetry
   Sleep studies
   Pulmonary function tests
           What can be done about PPS?

   Firstly, drugs don’t work, at least not the one’s
    we have at the moment.

   Modafanil and pyridostigmine, steroids and ivIg
    are all proven not to have any benefit.
               What can be Done for PPS?
                 Treat Co-Morbidities
   If you rely on your shoulders,
       protect them and seek early advice for shoulder symptoms.
        e.g.. “Save Our Shoulders”
       Insist on proper evaluation of the shoulder e.g. USS or MRI
       Ensure the surgeon has experience of PPS.
   Treat general medical and endocrine problems.
   Treat carpal tunnel syndrome
   Look at posture to prevent progressive deformities e.g..
    Profiling bed, trunk support when sitting.
   Make every effort to treat and avoid rising BMI: diet,
    Orlistat, Sibutramine.
Treat
Co-morbidities
Get  orthoses to off
load and support joints
that are failing

Use   lightweight
modern materials for
orthoses e.g. carbon
fibre, titanium
Treat
Co-Morbidities


Use strategies to
avoid over stressing
systems that are
already challenged
e.g. powered wheel
chair, PAPAW.
 Treat
 Co-Morbidities



Night time
hypoventilation can
be easily treated
with NIPPV
                Active Management of PPS

   Start an exercise program:
       Aerobic, i.e.. Within the limits of the muscles’ glucose
        and oxygen supplies. In practice this means 2-3 minutes
        exercise, 1-3 minutes rest.
       Within your limit (Avoid “boom and bust”). Do not
        exercise until it hurts the muscles. If your muscles ache
        and are stiff the next day you over did it.
       Use pacing and graded exercise goals: small increments
        in your limit are achievable e.g.. 5-10% every 1-2 weeks.
Exercise for PPS
Where    possible try
and use water based
activities: you are 30%
lighter in the water and
will off load joints that
might be struggling
with gravity based
exercises.

Be   consistent.

      reverses
Exercise
DECONDITIONING
             Active Management of PPS

   Get good pain control: non-steroidal anti-
    inflammatory drugs, medium grade opiates e.g..
    codeine, but use non-pharmacological means e.g..
    Counter stimulation TENS, rubifacients
   Keep warm, where possible, spend time in a warm
    climate (Nordby 2007)
   Keep respiratory difficulties under review and take
    advice about the need for night time ventilation
    support, stop smoking, and ask for advice about
    respiratory muscle training
Active Management
of PPS

Make   environmental
adaptations and use
assistive technology:
e.g.. Door entry
systems, remote
switches,
environmental control
systems, level access
bathroom facilities


Join   a group or start
one.
   Conclusion
People with PPS get
more out of their
muscles and joints than
would have been
expected.

They  seem to remain
independent in the long
term to a degree that is
contrary to
expectations.

The   symptoms are
manageable and with
proper measures quality
of life can remain good.