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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)
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Dr Michael Watt
Have I got it or have I got something else?
What can I do about it?
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)
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.
One nerve/motor muscle unit
multiple motor units /nerve
Infection of the polio survivor’s motor neuron by a different enterovirus
Loss of strength associated with aging, in already weakened musclesTheories (contd.)
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
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
Age of onset (More severe disease in adolescents and adults)
Severity of original paralysis
Managed with BiPAP
Other neuromuscular diseases
Spinal cord and nerve root problems
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
EMG and nerve conduction studies
Pulmonary function tests
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.
Get orthoses to off load and support joints that are failing
Use lightweight modern materials for orthoses e.g. carbon fibre, titanium
Use strategies to avoid over stressing systems that are already challenged e.g. powered wheel chair, PAPAW.
Night time hypoventilation can be easily treated with NIPPV
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.
Exercise reverses DECONDITIONING
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
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.
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.