Transcript Diagnosis and Medical Management of Post

Diagnosis and Medical
Management of
Post-Polio Syndrome
Dr Michael Watt
Consultant Neurologist
RVH, Belfast
How easy it is to Forget
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What is PPS?
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Have I got it or have I got something else?
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What can I do about it?
History of PPS
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First case described in 1875 (Raymond, 1875)
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Zilkha (1962) described 11 cases occurring 17-43 years post
acute illness.
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Halstead (1985) – “post polio syndrome”, (PPS), then, redefined it in 1991.
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Dalakas (1995) defined post polio muscular atrophy (PPMA)
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Berg(1996) “Post Polio Muscular dysfunction” (PPMD)
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Howard (1988,2003)) Post-polio functional deterioration
(PPFD)
Halstead’s 1985 Definition
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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:
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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
fatigue
7
2
1
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*
(N = 132)
Wisconsin§
(N = 79)
N
%
N
%
Fatigue
117
89
68
86
Muscle pain
93
71
68
86
Joint pain
93
71
61
77
Affected muscles
91
69
63
80
Unaffected
muscles
66
50
42
53
37
28
31
39
Walking
84
64
--
--
Climbing stairs
80
61
53
67
Dressing
23
17
13
16
HEALTH PROBLEMS
Weakness:
Atrophy
FUNCTIONAL
PROBLEMS
Epidemiology of PPS
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The frequency of PPS ranges between 15%80%, depending which population are studied,
and which criteria are applied.
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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:
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Remaining healthy motor
neurons can no longer
maintain new sprouts
Decompensation / chronic
denervation and
reinervation process.
Denervation exceeds
reinervation
Theories (contd.)
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Motor neuronal loss due to
reactivation of a persistent
latent virus.
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Infection of the polio
survivor’s motor neuron by
a different enterovirus
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Loss of strength
associated with aging, in
already weakened muscles
Possible Causes of Late
Complications of Polio
What Causes PPS?
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Accelerated natural ageing
Falling nerve to muscle motor unit ratio
Inflammation and active immune response
Co-morbidity:
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Orthopaedic problems
Radiculopathy and entrapment neuropathy
Respiratory failure
General medical problems
PPS is more likely with
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increasing age;
the more severe the initial weakness was
The more time that elapses after the attack of polio
Non-paralytic polio and PPS?
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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
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Fatigue (Commonest)
Weakness
Muscle pain
Gait disturbance
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Respiratory problems
Swallowing problems
Cold intolerance
Sleep apnoea
Fatigue
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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
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Disuse
Overuse
Inappropriate use
Chronic weakness
Weight gain
Joint problems
Muscle Pain
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Extremely prevalent in PPS
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Deep aching pain
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Myofascial pain syndrome / Fibromyalgia
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Small number of patients have muscle tenderness on
palpation
Swallowing Problems
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Can occur in bulbar and non bulbar polio
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Subclinical asymmetrical weakness in the
pharyngeal constrictor muscles : almost always
present in PPMA (Post polio muscular atrophy)
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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
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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
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Age of onset (More severe disease in
adolescents and adults)
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Severity of original paralysis
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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
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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?
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MRI scans
Blood tests
EMG and nerve conduction studies
X-rays
Overnight oximetry
Sleep studies
Pulmonary function tests
What can be done about PPS?
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Firstly, drugs don’t work, at least not the one’s
we have at the moment.
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Modafanil and pyridostigmine, steroids and ivIg
are all proven not to have any benefit.
What can be Done for PPS?
Treat Co-Morbidities
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If you rely on your shoulders,
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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
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Start an exercise program:
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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.
Exercise
reverses
DECONDITIONING
Active Management of PPS
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Get good pain control: non-steroidal antiinflammatory 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
one.
a group or start
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.