Diagnosis and Medical
Dr Michael Watt
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
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
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
Two or more of the following health problems occurring after
the stable period:
Muscle and or joint pain
New weakness in muscles previously affected or unaffected
New muscle atrophy
No other medical explanation found
Prospective study of New symptoms
NEW DIFFICULTIES EXPERIENCED BY SUBJECTS WITH PRIOR PARALYTIC
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
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
(N = 132) (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
66 50 42 53
Atrophy 37 28 31 39
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?
One nerve/motor muscle unit
multiple motor units /nerve
Remaining healthy motor
neurons can no longer
maintain new sprouts
Decompensation / chronic
Motor neuronal loss due to
reactivation of a persistent
Infection of the polio
survivor’s motor neuron by
a different enterovirus
Loss of strength associated
with aging, in already
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
Radiculopathy and entrapment neuropathy
General medical problems
PPS is more likely with
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
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
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
Generalized or muscular
Extremely prevalent in PPS
Deep aching pain
Myofascial pain syndrome / Fibromyalgia
Small number of patients have muscle tenderness on
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
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
Combination of the following:
Central: residual dysfunction of surviving bulbar
Obstructive: pharyngeal weakness and increased
musculoskeletal deformities from scoliosis or
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?
Is it PPS?
Spinal cord and
nerve root problems
Is it PPS? – Other things to think of
Other rheumatological disorders: rheumatoid
arthritis, lupus, Sjorgren’s syndrome or just
Endocrine disorders: hypothyroidism, adrenal
failure, rarely pituitary failure
Orthopaedic problems: shoulder rotator cuff tears
and impingement syndrome, spondylosis, bursitis,
Breathing disorders: restrictive problems with
scoliosis, obstructive sleep apnoea
General medical problems: heart failure, diabetes
How is it Investigated?
EMG and nerve conduction 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?
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,
Get orthoses to off
load and support joints
that are failing
modern materials for
orthoses e.g. carbon
Use strategies to
avoid over stressing
systems that are
e.g. powered wheel
be easily treated
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
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
adaptations and use
e.g.. Door entry
systems, level access
Join a group or start
People with PPS get
more out of their
muscles and joints than
would have been
They seem to remain
independent in the long
term to a degree that is
The symptoms are
manageable and with
proper measures quality
of life can remain good.