1. Diagnosis and Medical Management of Post-Polio Syndrome
Dr Michael Watt
Consultant Neurologist
RVH, Belfast

2. How easy it is to Forget

3. What is PPS?
Have I got it or have I got something else?
What can I do about it?

4. History of PPS First case described in 1875 (Raymond, 1875)
Zilkha (1962) described 11 cases occurring 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)

5. 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

6. 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
Pain alone 7§
Pain and fatigue
Weakness alone 4
Weakness and pain 9
Weakness and fatigue
Weakness, pain and fatigue 7
No new symptoms 18
* 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:

7. 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 %
HEALTH PROBLEMS
Fatigue
117 89 68 86
Muscle pain 93 71
Joint pain 61 77
Weakness:
Affected muscles 91 69 63 80
Unaffected muscles 66 50 42 53
Atrophy 37 28 31 39
FUNCTIONAL PROBLEMS
Walking 84 64 --
Climbing stairs 67
Dressing 23 17 13 16

8. 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.

9. What Causes PPS? Motor Neuron Loss?

10. One nerve/motor muscle unit
CNS
CNS
Normal:
One nerve/motor muscle unit
PPS:
multiple motor units /nerve

11. Pathophysiology Theories:
Remaining healthy motor neurons can no longer maintain new sprouts
Decompensation / chronic denervation and reinervation process.
Denervation exceeds reinervation

12. 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

13. Possible Causes of Late Complications of Polio

14. 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

15. 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

16. Main Clinical Features of PPS
Fatigue (Commonest)
Weakness
Muscle pain
Gait disturbance
Respiratory problems
Swallowing problems
Cold intolerance
Sleep apnoea

17. 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

18. Weakness Disuse Overuse Inappropriate use Chronic weakness Weight gain
Joint problems

19. Muscle Pain Extremely prevalent in PPS Deep aching pain
Myofascial pain syndrome / Fibromyalgia
Small number of patients have muscle tenderness on palpation

20. 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

21. 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

22. 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

23. Risk Factors for Sleep Apnoea
Age of onset (More severe disease in adolescents and adults)
Severity of original paralysis
Managed with BiPAP

24. Is it PPS?
Other neuromuscular diseases
Nerve entrapment

25. Is it PPS?
Spinal cord and nerve root problems
Scoliosis

26. 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

27. How is it Investigated?
MRI scans
Blood tests
EMG and nerve conduction studies
X-rays
Overnight oximetry
Sleep studies
Pulmonary function tests

28. 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.

29. 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.

30. 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

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

32. Treat Co-Morbidities
Night time hypoventilation can be easily treated with NIPPV

33. 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 % every 1-2 weeks.

34. 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

35. 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

36. 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.

37. 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.