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

Diagnosis and Medical Diagnosis and Medical Management of Management of

Post-Polio SyndromePost-Polio SyndromeDr Michael WattDr Michael Watt

Consultant NeurologistConsultant Neurologist

RVH, BelfastRVH, Belfast

How easy it is to ForgetHow easy it is to Forget

What is PPS?What is PPS?

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

What can I do about it?What can I do about it?

History of PPSHistory of PPS First case described in 1875 (Raymond, 1875)First case described in 1875 (Raymond, 1875)

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

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

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

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

Howard (1988,2003)) Post-polio functional Howard (1988,2003)) Post-polio functional deterioration (PPFD)deterioration (PPFD)

Halstead’s 1985 DefinitionHalstead’s 1985 Definition Confirmed history of polioConfirmed history of polio Partial or fairly complete neurological and Partial or fairly complete neurological and

functional recovery after the acute episode.functional recovery after the acute episode. Period of at least 15 years with neurological Period of at least 15 years with neurological

and functional stabilityand functional stability Two or more of the following health problems Two or more of the following health problems

occurring after the stable period:occurring after the stable period: Extensive fatigueExtensive fatigue Muscle and or joint painMuscle and or joint pain New weakness in muscles previously affected or New weakness in muscles previously affected or

unaffectedunaffected New muscle atrophyNew muscle atrophy Functional lossFunctional loss Cold intoleranceCold intolerance

No other medical explanation foundNo other medical explanation found

Prospective study of New symptomsProspective study of New symptomsNEW DIFFICULTIES EXPERIENCED BY SUBJECTS WITH PRIOR NEW DIFFICULTIES EXPERIENCED BY SUBJECTS WITH PRIOR

PARALYTIC POLIOPARALYTIC POLIO

   PatientsPatients

SymptomSymptom No.No.Requiring New Requiring New

AidsAidsChanging Changing ActivitiesActivities

Fatigue aloneFatigue alone 22 11 00

Pain alonePain alone 77§§ 00 11

Pain and fatiguePain and fatigue 11 11 00

Weakness aloneWeakness alone 44 00 00

Weakness and Weakness and painpain

99 22 00

Weakness and Weakness and fatiguefatigue

22 11 00

Weakness, pain Weakness, pain and fatigueand fatigue

77 22 11

No new No new symptomssymptoms

1818 00 00

** Types of complaints reported by 32 of the 50 subjects with paralytic polio. Twenty-two complained of some new 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 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.cases, the symptoms had led to lifestyle changes.§§ All seven complained of nonradiating lumbar or cervical pain.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-Windebank AJ et al. Late effects of paralytic poliomyelitis in Omsted County, Minnesota. Neurology. 1991; 41:507-507507

MOST COMMON NEW HEALTH AND FUNCTIONAL PROBLEMS OF MOST COMMON NEW HEALTH AND FUNCTIONAL PROBLEMS OF PATIENTS WITH CONFIRMED POLIO EVALUATED IN TWO POST-POLIO PATIENTS WITH CONFIRMED POLIO EVALUATED IN TWO POST-POLIO

CLINICSCLINICS

  TexasTexas

(N = 132)(N = 132)WisconsinWisconsin§§(N = 79)(N = 79)

   NN %% NN %%

HEALTH HEALTH PROBLEMSPROBLEMS

FatigueFatigue 117117 8989 6868 8686

Muscle painMuscle pain 9393 7171 6868 8686

Joint painJoint pain 9393 7171 6161 7777

Weakness:Weakness:         

  Affected Affected musclesmuscles

9191 6969 6363 8080

  Unaffected Unaffected musclesmuscles

6666 5050 4242 5353

AtrophyAtrophy 3737 2828 3131 3939

FUNCTIONAL FUNCTIONAL PROBLEMSPROBLEMS

WalkingWalking 8484 6464 ---- ----

Climbing stairsClimbing stairs 8080 6161 5353 6767

DressingDressing 2323 1717 1313 1616

Epidemiology of PPSEpidemiology of PPS

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

In European populations a prevalence In European populations a prevalence of between 46% (Holland) and 60% of between 46% (Holland) and 60% (Edinburgh, Norway, Denmark) is (Edinburgh, Norway, Denmark) is seen in the literature.seen in the literature.

What Causes PPS?What Causes PPS?Motor Neuron Loss?Motor Neuron Loss?

CNSCNS

Normal: One nerve/motor muscle unit

PPS: multiple motor units /nerve

PathophysiologyPathophysiology

TheoriesTheories:: Remaining healthy Remaining healthy

motor neurons can motor neurons can no longer maintain no longer maintain new sproutsnew sprouts

Decompensation / Decompensation / chronic denervation chronic denervation and reinervation and reinervation process.process.

Denervation exceeds Denervation exceeds reinervationreinervation

Theories (contd.)Theories (contd.) Motor neuronal loss Motor neuronal loss

due to reactivation of due to reactivation of a persistent latent a persistent latent virus.virus.

Infection of the polio Infection of the polio survivor’s motor survivor’s motor neuron by a different neuron by a different enterovirusenterovirus

Loss of strength Loss of strength associated with aging, associated with aging, in already weakened in already weakened musclesmuscles

Possible Causes of Late Possible Causes of Late Complications of PolioComplications of Polio

What Causes PPS?What Causes PPS?

Accelerated natural ageingAccelerated natural ageing Falling nerve to muscle motor unit ratioFalling nerve to muscle motor unit ratio Inflammation and active immune responseInflammation and active immune response Co-morbidity:Co-morbidity:

Orthopaedic problemsOrthopaedic problems Radiculopathy and entrapment neuropathyRadiculopathy and entrapment neuropathy Respiratory failureRespiratory failure General medical problemsGeneral medical problems

PPS is more likely with PPS is more likely with increasing age; increasing age; the more severe the initial weakness was the more severe the initial weakness was The more time that elapses after the attack of polioThe more time that elapses after the attack of polio

Non-paralytic polio and PPS?Non-paralytic polio and PPS?

For non-paralytic polio it is impossible to For non-paralytic polio it is impossible to exclude a scaled down version of the exclude a scaled down version of the same processes.same processes.

Such a diagnosis however is Such a diagnosis however is presumptive and cannot be categorically presumptive and cannot be categorically confirmed.confirmed.

When we have further knowledge about When we have further knowledge about the specificity and sensitivity of EMG, the specificity and sensitivity of EMG, muscle biopsy and immunological tests it muscle biopsy and immunological tests it should be possible to give more definite should be possible to give more definite diagnosesdiagnoses

Main Clinical Features of Main Clinical Features of PPSPPS

Fatigue Fatigue (Commonest)(Commonest)

WeaknessWeakness Muscle painMuscle pain Gait disturbanceGait disturbance

Respiratory Respiratory problemsproblems

Swallowing Swallowing problemsproblems

Cold intoleranceCold intolerance Sleep apnoeaSleep apnoea

FatigueFatigue Prominent in the early hours of the Prominent in the early hours of the

afternoonafternoon Decreases with restDecreases with rest Pathogenesis:Chronic pain / Muscle painPathogenesis:Chronic pain / Muscle pain Sleep disorders/ respiratory dysfunctionSleep disorders/ respiratory dysfunction Difficulty in remembering/ concentratingDifficulty in remembering/ concentrating Decreased muscular endurance / Decreased muscular endurance /

Increased muscular fatigabilityIncreased muscular fatigability ““Polio wall”Polio wall” Generalized or muscularGeneralized or muscular

WeaknessWeakness

Disuse Disuse Overuse Overuse Inappropriate useInappropriate use Chronic weaknessChronic weakness Weight gainWeight gain Joint problemsJoint problems

Muscle PainMuscle Pain

Extremely prevalent in PPSExtremely prevalent in PPS

Deep aching painDeep aching pain

Myofascial pain syndrome / FibromyalgiaMyofascial pain syndrome / Fibromyalgia

Small number of patients have muscle Small number of patients have muscle tenderness on palpationtenderness on palpation

Swallowing ProblemsSwallowing Problems

Can occur in bulbar and non bulbar Can occur in bulbar and non bulbar poliopolio

Subclinical asymmetrical weakness Subclinical asymmetrical weakness in the pharyngeal constrictor in the pharyngeal constrictor muscles : almost always present in muscles : almost always present in PPMA (Post polio muscular atrophy)PPMA (Post polio muscular atrophy)

Not all are symptomaticNot all are symptomatic

Cold IntoleranceCold Intolerance

Autonomic nervous system dysfunction?Autonomic nervous system dysfunction?

May relate to sympathetic May relate to sympathetic intermediolateral column damage intermediolateral column damage during acute poliomyelitisduring acute poliomyelitis

Peripheral component may include Peripheral component may include muscular atrophy leading to reduced muscular atrophy leading to reduced heat productionheat production

Sleep ApnoeaSleep Apnoea

Combination of the following:Combination of the following: Central: residual dysfunction of surviving Central: residual dysfunction of surviving

bulbar reticular neuronsbulbar reticular neurons Obstructive: pharyngeal weakness and Obstructive: pharyngeal weakness and

increased musculoskeletal deformities increased musculoskeletal deformities from scoliosis or emphysemafrom scoliosis or emphysema

PPMA, diminished muscle strength of PPMA, diminished muscle strength of respiratory,intercostal & abdominal respiratory,intercostal & abdominal muscle groupsmuscle groups

Risk Factors for Sleep Risk Factors for Sleep ApnoeaApnoea

Age of onset (More severe disease Age of onset (More severe disease in adolescents and adults)in adolescents and adults)

Severity of original paralysisSeverity of original paralysis

Managed with BiPAPManaged with BiPAP

Is it PPS?Is it PPS?

Other Other neuromuscular neuromuscular diseasesdiseases

Nerve Nerve entrapmententrapment

Is it PPS?Is it PPS?

Spinal cord Spinal cord and nerve root and nerve root problemsproblems

ScoliosisScoliosis

Is it PPS? – Other things to think Is it PPS? – Other things to think ofof

Other rheumatological disorders: Other rheumatological disorders: rheumatoid arthritis, lupus, Sjorgren’s rheumatoid arthritis, lupus, Sjorgren’s syndrome or just osteoarthritissyndrome or just osteoarthritis

Endocrine disorders: hypothyroidism, Endocrine disorders: hypothyroidism, adrenal failure, rarely pituitary failureadrenal failure, rarely pituitary failure

Orthopaedic problems: shoulder rotator Orthopaedic problems: shoulder rotator cuff tears and impingement syndrome, cuff tears and impingement syndrome, spondylosis, bursitis, metatarsalgia.spondylosis, bursitis, metatarsalgia.

Breathing disorders: restrictive problems Breathing disorders: restrictive problems with scoliosis, obstructive sleep apnoeawith scoliosis, obstructive sleep apnoea

General medical problems: heart failure, General medical problems: heart failure, diabetesdiabetes

How is it Investigated?How is it Investigated?

MRI scansMRI scans Blood testsBlood tests EMG and nerve conduction studiesEMG and nerve conduction studies X-raysX-rays Overnight oximetryOvernight oximetry Sleep studiesSleep studies Pulmonary function testsPulmonary function tests

What can be done about PPS?What can be done about PPS?

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

Modafanil and pyridostigmine, Modafanil and pyridostigmine, steroids and ivIg are all proven not steroids and ivIg are all proven not to have any benefit.to have any benefit.

What can be Done for PPS?What can be Done for PPS?Treat Co-MorbiditiesTreat Co-Morbidities

If you rely on your shoulders, If you rely on your shoulders, protect them and seek early advice for shoulder protect them and seek early advice for shoulder

symptoms. e.g.. “Save Our Shoulders”symptoms. e.g.. “Save Our Shoulders” Insist on proper evaluation of the shoulder e.g. Insist on proper evaluation of the shoulder e.g.

USS or MRI USS or MRI Ensure the surgeon has experience of PPS.Ensure the surgeon has experience of PPS.

Treat general medical and endocrine problems.Treat general medical and endocrine problems. Treat carpal tunnel syndromeTreat carpal tunnel syndrome Look at posture to prevent progressive Look at posture to prevent progressive

deformities e.g.. Profiling bed, trunk support deformities e.g.. Profiling bed, trunk support when sitting.when sitting.

Make every effort to treat and avoid rising Make every effort to treat and avoid rising BMI: diet, Orlistat, Sibutramine.BMI: diet, Orlistat, Sibutramine.

Treat Treat Co-morbiditiesCo-morbidities

Get orthoses to Get orthoses to off load and off load and support joints support joints that are failingthat are failing

Use lightweight Use lightweight modern materials modern materials for orthoses e.g. for orthoses e.g. carbon fibre, carbon fibre, titaniumtitanium

Treat Treat Co-MorbiditiesCo-Morbidities

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

Treat Treat Co-MorbiditiesCo-Morbidities

Night time Night time hypoventilation hypoventilation can be easily can be easily treated with treated with NIPPVNIPPV

Active Management of PPSActive Management of PPS

Start an exercise program:Start an exercise program: Aerobic, i.e.. Within the limits of the muscles’ Aerobic, i.e.. Within the limits of the muscles’

glucose and oxygen supplies. In practice this glucose and oxygen supplies. In practice this means 2-3 minutes exercise, 1-3 minutes rest. means 2-3 minutes exercise, 1-3 minutes rest.

Within your limit (Avoid “boom and bust”). Within your limit (Avoid “boom and bust”). Do not exercise until it hurts the muscles. If Do not exercise until it hurts the muscles. If your muscles ache and are stiff the next day your muscles ache and are stiff the next day you over did it.you over did it.

Use pacing and graded exercise goals: small Use pacing and graded exercise goals: small increments in your limit are achievable e.g.. increments in your limit are achievable e.g.. 5-10% every 1-2 weeks.5-10% every 1-2 weeks.

Exercise for Exercise for PPSPPSWhere possible try Where possible try and use water and use water based activities: based activities: you are 30% lighter you are 30% lighter in the water and in the water and will off load joints will off load joints that might be that might be struggling with struggling with gravity based gravity based exercises.exercises.

Be consistent.Be consistent.

Exercise reverses Exercise reverses DECONDITIONINGDECONDITIONING

Active Management of PPSActive Management of PPS

Get good pain control: non-steroidal anti-Get good pain control: non-steroidal anti-inflammatory drugs, medium grade opiates inflammatory drugs, medium grade opiates e.g.. codeine, but use non-pharmacological e.g.. codeine, but use non-pharmacological means e.g.. Counter stimulation TENS, means e.g.. Counter stimulation TENS, rubifacientsrubifacients

Keep warm, where possible, spend time in a Keep warm, where possible, spend time in a warm climate (Nordby 2007)warm climate (Nordby 2007)

Keep respiratory difficulties under review Keep respiratory difficulties under review and take advice about the need for night and take advice about the need for night time ventilation support, stop smoking, and time ventilation support, stop smoking, and ask for advice about respiratory muscle ask for advice about respiratory muscle trainingtraining

Active Active Management of Management of PPSPPS

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

Join a group or Join a group or start one.start one.

ConclusionConclusionPeople with PPS People with PPS get more out of get more out of their muscles and their muscles and joints than would joints than would have been expected. have been expected.

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

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