recognizing the impacts of poliomyelitis in children and planning the rehabilitation strategies

8/14/2019 Recognizing the Impacts of Poliomyelitis in Children and Planning the Rehabilitation Strategies

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RECOGNIZING THE IMPACTS OF

POLIOMYELITIS IN CHILDREN ANDPLANNING THE REHABILITATION

SRATEGIES

Ronald E. Pakasi, MD

Physical Medicine & Rehabilitation Dept.

Jakarta, Indonesia



History

• First recorded symptomssimilar to poliomyelitis:Egyptian stele datingfrom the EighteenthDynasty (1580-1350

B.C.)⇒”a crippled young man,

apparently a priest,with a withered &shortened left leg, witha foot positioned typicalof flaccid paralysis,

using his staff forsupport”



Polio - Timeline



Epidemiology

• Indonesia: 2

outbreaks in the

past decade

• 1995

• 2005

• Any ages:

neonates > severe

• M : F ratio = 1 : 1



Etiology - The Virus -

• Picornaviridae(enterovirus) - RNA virus

• Enterically infecting virus• Food- / water-borne

• Fecal-oral / oral–oral /

respiratory transmission

• Initial replication:

oropharynx• Main invasion site: small

intestine

• Best temp.: 370 C



Etiology – The Vaccine -

The Vaccine • Sabin-live oral polio

vaccine (OPV)

• Widely used in 3rd worldcountries

• Vaccine-associatedparalytic poliomyelitis



Pathophysiology

• Replication of 1 cycle: + 8hours

• RNA core: capsid proteins → allows binding to specific cellmembrane components

(gateway)• 1 hour post infection (PI):

margination of chromatin

(accumulate within thenuclear envelope)

• 2.5 – 3 hours PI: membranous

vesicles emerge → spreadoutward



Pathophysiology

• Replication (pharynx ⇒

small intestine): first 1-3

weeks of incubation• Incubation: host-to-host

transmission possible

• Replication detectable:

24-72 hours after virus

ingestion• ± 5% pts: nervous system

involvement



P l ti



Paralytic Poliomyelitis

• Spinal poliomyelitis

• Bulbar poliomyelitis• Bulbospinal

poliomyelitis• Polioencephalitis



Paralyt ic Pol iomyel i t is

• 2-5% of polioviral infection cases

• Spinal cord anterior horn cells & othermotor neurons: selectively susceptible

• Incubation: 4-10 days ⇒ may extend to 4-

5 weeks

• 1st symptoms: nonspecific fever & muscle

weakness (↓↓ >2-3 days)

• Abrupt onset of asymmetric flaccid

paralysis



Spinal Pol iomyel i t is

• Affects LE > UE > Trunk

• Preceded by: ↑↑ muscle pain & spasm

• Paralysis: asymmetric, flaccid, patchy, proximal > distal⇒ may extent to other limbs after > 5–6 days

• Paralysis⇒ max in 48 hrs (some: > 1 wk)• Paralysis progression: ↓↓ if the fever (-) in 48 hrs

• Reflexes ↓↓ ⇒ (-) & bulbar involvement may follow

• Muscle atrophy: > 3 wks (max 12-15 wks ⇒ permanent)• Paresthesia: frequent w/o sensory dist.

• Rare accompanying conditions: trans. myelitis +

paraparesis, urinary ret., sensory & autonomic dist.



Bulbar Pol iomyel i t is

• A.K.A. brainstem poliomyelitis (10-15% of paralyticpoliomyelitis cases)

• Isolated bulbar w/o limb weakness: more commonin children (especially w/ history of tonsils &adenoids removal)

• Adult type: bulbospinal poliomyelitis

• Pain, nuchal rigidity & hypertonia: brainstem,spinal ganglia, & posterior column

• Cranial nerves: VII, IX, X, (rare: III)⇒ facialweakness, dysphagia, dysphonia, & oculomotor

palsy (rare)

• Reticular formation: cardiorespiratory problems(discussed later)



Polioencephalitis

• Very rare & uncommonly occurs w/o paralysis

• Paralysis: may emerges 24-48 hrs > initial onset• Clinical manifestations: tremulousness,

obtundation, agitation, & autonomic dysfunction

• Autonomic: labile hypertension / hypotension,tachycardia, arrhythmias, & hyperhydrosis

• UMN signs: spasticity, hyperreflexia, & Babinski• Other signs: muscle pains, muscle cramps,

fasciculation, & radicular pain



Diagnostic Procedures

• Lab: CSF (pleocytosis, ↑

protein level)

• Lab: CBC, culture (stool, blood, etc.)

• EMG

• MRI



FUNCTIONAL ALTERATIONS



Neuromusculoskelet al Funct ion

AFP in polio: 3 phases (“triad two”)

• 1st: acute ⇒ subacute phase (1st 2 wks⇒ 2 wks to 2 mo)

• 2nd : convalescent phase (>2 mo - 2 yrs)• 3rd : chronic / residual phase (> 2 yrs)

→ post polio condition→ → post polio syndromepost polio syndrome




• Functional alterations : observed in mobility &

gait function• UE paralysis: major predilection site → deltoids

& triceps brachii ⇒

object manipulations may

be normal

• Accompanying symptom of pain: devastating &

may disturb other functions as well

• Pain: muscles, joints, & nerves ( >> lower back)

• Hyperesthesia ⇒ pain ↑↑




• Children PE: transitional mobility activities& walking activity

• Tests: standing on toes, standing on heels,squat-to-stand test, sit-to-stand test, jump

in one leg, & walk test• Infants PE: observe behavior on supineposition

• Paralysis: frog-leg position

• Other tests: sharp stimulus test &

pendulum test



Musculoskelet al Compl icat ions




Chi ldren wit h pol io: are st i l l in t hei r development al & growing age !!

Impact s of paralysis on a growing l imb:

• shortening & ↓↓ in diameter of the bone, due to:

• << muscle pull + vascular supply disturbance + ↓↓ nervestimulation

• Bone will become << compact & may not able tosupport loading

• Fractures may easily develop

• Bone-shortening in LE: result in LLD ⇒ disturb theposture balance on the vertebrae ⇒deformity in

vertebrae (scoliosis)





M l k l t l C l i t i




C di l F t i



Cardiopulmonary Funct ion

• Respiratory function → triple threat:weak respiratory muscles, poor cough

efforts & risk of food aspiration• Intercostal paralysis: AHC infection in

thoracic region

• Diaphragm paralysis: AHC infection in themid-cervical region

• Involvements in: dorsal nuclei of theVagus & the medial reticular nuclei(vasomotor nuclei): pulmonary edema,

CO2 retention, and papilledema



Cardiopulmonary Funct ion

• Involvement of N. Vagus: dysphagia ⇒ damaged

innervation to the larynx ⇒ potential airwayobstruction (early tracheotomy

• Paralysis of the larynx: saliva / mucus go down

to the lower respiratory tract ⇒ pulmonaryinfection / edema

• Paralysis of the soft palate: speech disturbance

• Medullary / hypothalamus involvements: 2nd

hypertension



Behavioral Problems

• Tendencies in polio survivors: develop

behavioral changes• Type A personality: non-compliant to

rehabilitation program, especially vocational

• Characteristic: hard-driving and excessiveworker, pressured, easily angered,

perfectionist, too sensitive to criticism &

failure, hyperactive; have a chronic insecurity

feeling, & low self-esteem

GENERAL REHABILITATION



GENERAL REHABILITATION

MANAGEMENT IN POLIOMYELITIS

•ACUTE PHASE

•CONVALESCENT PHASE•RESIDUAL PHASE



ACUTE PHASE

• Physiatrist: determine whether there are

any bulbar involvements or not• Respiratory assistance ? (life-saving

intervention)

• Infants with respiratory failure : negative

extrathoracic pressure ventilation (NEPV)

• Older children: intubation / intermittent

positive pressure ventilation



ACUTE PHASE

• Bulbar involvement: inability of the

patient to swallow (“drown” in his/herown secretions)

• Management: respiratory assistance +positioning technique

Respi rat ory Assist ance + Posi t ioning



Respi rat ory Assist ance + Posi t ioning

Technique



ACUTE PHASE

• Bulbar palsy w/o respiratory failure: risk

of aspiration (+) ⇒ owing to dysphagia• Emergency management: posturing

technique using a medially-angulated bed+ pillows to support the center of the

mattress

• Secretions will trickle down from the

patient’s mouth ⇒ aspiration risk can be

prevented



ACUTE PHASE



ACUTE PHASE

Other rehabilitation

strategies:

• preventing

complications

secondary toprolonged bed rest /

immobilization

• pain management

ACUTE PHASE P i M



ACUTE PHASE – Pain Management

Medications and supported by physical

modalities:• TENS

• Hot pack (Canny pack)• Infra red therapy

• Electrical stimulation

ACUTE PHASE E l M bi l i i



ACUTE PHASE – Ear ly Mobi l izat ion

Goals of ear ly mobi l izat ion:

• maintaining & exercising movementcoordination

• preventing pressure ulcers• prevention of deconditioning syndrome

⇒ (takes place after the febrile (-) &

progression of paralysis ↓↓)

ACUTE PHASE E l M bi l i t i



ACUTE PHASE – Ear ly Mobi l izat ion

• Bed mobilization: rolling, side-lying,

prone, sitting, or standing – using tiltingtable

• Crawling activity: if possible• Abdominal breathing exercise + chest

expansion exercise: as early as possible

• Sitting balance exercise: if possible

(preparation for ambulation)



CONVALESCENT CONVALESCENT

PHASE PHASE

CONVALESCENT PHASE



CONVALESCENT PHASE

• Hydrotherapy

• Muscle strengthening exercise (affected& unaffected limbs)

• Standing & gait preparation• Lower orthosis / spinal orthosis



CONVALESCENT PHASE



RESIDUAL PHASE



RESIDUAL PHASE

Hallmark of rehabilitation: develop substitutionpattern of the muscles (enabling patient to

perform ADLs maximally)• Orthosis: mandatory (exc. complete recovery)

• Operative management: sometimes necessary

Evaluation of the patient:

• 1st 2 months: every week (mo-1) & every 2weeks (mo-2)

• > 2-6 months: evaluate every 2 months

• > 2 – 3 years: evaluate every 6 months



PART II



PART IIPART II

POSTPOST--

REHABILITATIONREHABILITATION

MANAGEMENTMANAGEMENT



AN APPROACH TO THE PATIENT WITH

SUSPECTED POST POLIO SYNDROME



SUSPECTED POST POLIO SYNDROME

PRIME SYMPTOMS

• Fatigue, pain, & weakness: almost alwayspresent

• Fatigue (89%); pain in muscle / joint (86%);

new weakness (83%) in previously symptomatic(69%) / asymptomatic (50%) muscles

• New Atrophy (28%) ⇒~ Post Polio Muscular

Atrophy (PPMA)• ADL difficulties (78%) = functional loss

⇒

walking (64%); climbing stairs (61%);

dressing (17%)

ADDITIONAL PRESENTING PROBLEMS



ADDITIONAL PRESENTING PROBLEMS

• Pulmonary dysfunction

• Sleep Disorders• Dysphagia

• Cold intolerance• Degenerative arthritis

• Social and psychological problems

IDENTIFY AREAS OF DYSFUNCTION



• History ⇒ identifying: fatigue, dysphagia, sleepdisorders, & ADL alterations

• Neurologic exam: atrophy / weakness, reflexesare not ↑↑

• Observe the "good" l imb : (+) significant

weakness w/o patient’s awareness• Leg muscles: use functional tests [MMT may notdetect ↓↓ quadriceps strength to 30% of normal→ although sufficient for routine ADLs]

• MMT: use mechanical advantage ⇒ (e.g. mm.triceps / quadriceps with the elbow / knee

flexed >900

; m. psoas in supine position)





• General PE & biomechanical exam: note

obesity, joint deformity, overusesyndromes, & scoliosis

• EMG: document previous AHC disease

(especially if previous polio is doubtful) &

ruling out other neuromuscular pathologies

/ identifying subclinically involved muscles• Lab: CK ↑ (may not correlate with

progressive weakness)



REHABILITATION

STRATEGIES



Management of PPS



Management of PPS

3. Management of fatigue

• Energy conservation techniques• Lifestyle changes

• Pacing• Regular rest periods, naps during day

• Meds: amitriptyline (improve sleep) /pyridostigmine (?? ⇒ trial in progress)



Management of PPS



4. Management of pain

A. Post-polio muscular pain, muscular cramps,

muscular pain with activity• Activity reduction

• Pacing (rest periods during activity)

• Moist heat, ice, and stretching• Use of assistive devices

• Life style modifications

B. Fibromyalgia

• Meds: amitriptyline / cyclobenzaprine

• Aerobic exercise

Management of PPS



4. Management of pain

C. Joint and soft tissue abnormalities

• Modification of extremity use• Physiotherapy for use of physical modalities,

strengthening, stretching.

• Orthoses / other assistive devices

• Meds: NSAIDs, steroid injections / Surgery

D. Treatment of other superimposed neurologicdisorders (e.g. CTS, radiculopathy, spinal

stenosis)

Management of PPS



g

5. Pulmonary dysfunction

• Preventive measures (pneumococcalvaccine, yearly influenza vaccine)

• Ventilatory assistance (non-invasive

methods preferred)

• Identification & treatment of sleep apnea

• Glossopharyngeal breathing

Management of PPS



g

6. Dysphagia

• Changing or restricting diet• Use of special breathing techniques

• Use of special swallowing techniques• Monitoring fatigue, & taking larger meals

earlier, & smaller meals later• Avoiding eating when fatigued

Management of PPS



g

7. Psychosocial difficulties

• Interdisciplinary approach• Post-polio support group

• Evaluation and treatment by socialworkers, psychologists, psychiatrists

Lifestyle Modifications



• The "sine qua non" of all attempts atsuccessful management of PPS

• Patients: desperate, have a belief toovercome their disability through the "no pain, no gain" approach

• “No pain, no gain“ approach: may besuccessful after the acute onset ⇒ now

become self destructive• Persistence in this approach: spiral of

deteriorating function & ↓↓

self worth

Lifestyle Modifications



• Understand the concept of "living with"

PPS !!

• Understanding the need for lifestyle

modification: rarely achieved at 1st visit→ must be reintroduced by an OT/PT and

reinforced & monitored at subsequentphysician visits

Increase Muscle Capacity



a) Achieving ↑↑ strength / endurance

• Strength: isometric exercise ⇒selectmuscles carefully (some muscles will

already be functioning at their maximum)

• Exercise: may have a deleterious effect

by forcing these muscles beyond their

metabolic capacity & producing injury




b) Appropriate exercise: ↑↑ endurance, ↓↓susceptibility to fatigue, ↓↓ long term

deterioration• Initial difficulties: occur in almost allpatients (from overdoing?)

• May tend to associate fatiguing ADLs(challenge the weakest musculature & do

not provide an effective aerobic traininglevel) with exercise

⇒

can be an instructive opportunity for

understanding the “lifestyle modification"

Goals in aerobic exercise:



– Educate the patient to avoid potentiallyharmful exercise-induced fatigue

⇒ Strategy: establish the level of peakperformance by patient history → start at50% of peak performance & slowly ↑↑

performance as tolerated– Select exercises which can create a training

effect in the patient with weakened,atrophic musculature & overuse syndromes

⇒ Strategy: exercise intervals withintervening rests, just as is pacing of ADLs




c) Use of bracing, orthotics, or other aids which extend,amplify or substitute for muscles (to ↑ musclecapacity)

d) Pharmacologic treatment of fatigue: (↑↑ fatiguethreshold) ⇒ still observed & needs confirmation:

– Amantadine: up to 100 mg BID as tolerated

– Deprenyl: up to 5 mg BID as tolerated

– Mestinon: up to 60 mg TID (needs carefulmonitoring!)

Medications: just the aids to give a running start forthe rehabilitation process (patient shouldunderstands them as a non curative treatment)

Decrease Muscle Load To LessThan Muscle Capacity



a) PACING of activity

• Implementation: patients must identify each of

the ADL’s length of time (before experiencingfatigue)

• Break up activities into smaller modules of time(each with << duration (before fatigue occurs)

• Concept of PACING: ENERGY BUDGETINGENERGY BUDGETING ⇒ patient has a fixed energy expenditure (EE) foreach day & should be "spent" on the highest

priority of ADLs• Concept of exceeding use of this daily limit:

acquire debt ⇒

correlates to metabolic injury of

the motor unit (overuse)

Decrease Muscle Load To Less

Than Muscle Capacity



p y

b) Other means of decreasing muscle load:

• diet (for overweight >>)• orthotics (improve mechanical efficiency)

• wheelchairs / scooters to save EE• treatment of chronic overuse syndromes

Treat Specific Complications



• a) Attention to specific complications: e.g. dysphagia,pulmonary dysfunction & sleep disturbances

• b) Functional consequences resulting from overusesyndromes (⇒ joint deformity)

→ Physiatry consultation / orthopedic intervention

→ Orthotic prescriptions (i.e., splints, braces, AFO's)• c) Somatization, depression, anxiety, & self worth

problems: may occur as capacity ↓↓ (referral forcounseling or polio support group)

• d) Evaluate and/or modify work duties through referralto OT or vocational counselor

Exercise in PPS Patients



• Include: isotonic, isokinetic and isometricstrengthening exercise programs + aerobic

exercise• Average level of aerobic fitness in post-polio

patients: 5.6 METS (similar to MCI patients)

• Aerobic exercise (UE + LE): bicycle ergometeror treadmill

• Bicycle ergometer: improved VO2max, duration

of exercise, watts attained during exercise, andmaximum expired volume per unit time

• Treadmill training: improved economy orenergy cost of walking (+ walking duration)




Exercise prescription: dependent on several factors:

• Isometric: MMT < 3 or in muscles over a painful joint

• Isotonic: MMT > 3 and without a painful joint• Aerobic exercise program: bicycle ergometer, walking,

or swimming (should be an activity which the patientenjoys to increase compliance)

• Warm-up & cool-down period + stretching exercisesshould be included

• Early in the exercise program: monitored carefully (to

ensure that the exercise prescription is being followedcorrectly & muscles and joints overuse is not occurring)

• Encourage self-monitor




THANK YOUTHANK YOU



POST PRESENTATION

NOTES…

Pathophysiology of PPS



• EXTENSIVE NEURONAL INVOLVEMENT IN

THE ACUTE POLIO INFECTION• MOTOR UNIT REMODELING IN THE POST

RECOVERY PHASE

• DECOMPENSATION THEN PRODUCES

POST POLIO SYNDROME

EXTENSIVE NEURONAL INVOLVEMENT IN THE ACUTE POLIO INFECTION



• Infection is >> widespread than anterior horn cells (AHC)

• AHC infection: largely subclinical (⇒ residual capacity of uninfected & surviving neurons)

• Infection outside AHC: largely subclinical (may contributeto the disabling symptoms of fatigue & pain ⇒ physiologic basis is uncertain)

• 95% of motor neurons: infected in an average acuteinfection (50% neuronal fatality rate)

• Segmental involvement ↑↑ ( causing lack of weaknesssymmetry)

• Other involvements: intermediolateral horns & dorsal rootganglia, motor cortex, hypothalamus, globus pallidus,brainstem nuclei, reticular formation, cerebellar roof

nuclei, & vermis.

MOTOR UNIT REMODELING IN THE POST

RECOVERY PHASE



RECOVERY PHASE

• Example of muscles: quadriceps (N) has ± 200 musclefibers/ AHC {AHC (N) can adopt ± 1,000 orphanedmuscle fibers}

• > 50 % motor units may be lost w/o symptoms

(Walking (N): only 15-20% of max muscle strength)• Clinical improvement: through recovery of mildly

affected neurons, collateral sprouting, &

strengthening (hypertrophy) of intact musculature• ↑↑ demand on surviving motor units: ⇒ ↑↑ firing

frequency ⇒ change in fiber type to predominantlyaerobic "slow twitch" fibers with ↑↑

vascularity

DECOMPENSATION THEN PRODUCES POST POLIO SYNDROME



• ↑↑ metabolic burden on surviving AHC (includingasymptomatic muscles) as they direct more muscle

fibers to contract→ often to achieve the same forceof contraction

• ⇒⇒ AHC fatigue ⇒⇒ premature metabolic injury(even cell loss)

• Fatigued neurons: unable to continue to trophicallysupport as many muscle fibers ⇒⇒ collateral sproutsto some muscle fibers will degenerate ⇒⇒ strength

will be lost to the motor unit ⇒⇒ spiral of decline !!• This mode of decompensation (+ fatigue) ⇒ AHC

based (not a static process → may be dynamicdenervation & reinnervation)




Anot her mode of decompensat ion is muscle f iber based

• Rapidly firing muscle fibers ⇒⇒ ↑

lactic acid (not be

adequately dissipated) ⇒ especially true withisometric contraction ⇒ muscle fiber fatigue ⇒⇒muscle fiber injury ⇒ lost function ⇒ spiral of decline !!

• ↑↑ mechanical load (e.g. from ↑↑ weight or ↑↑ physical activity) or ↓↓

force generating capacity

(e.g. from inactivity following illness / injury) ⇒⇒met abol ic f ailure in motor units / muscle fibersfunctioning close to their capacity


Th l i l i k j i &



• The resulting relative weakness ⇒⇒ joint &muscle misuse & overuse ⇒⇒ arthritis &overuse syndromes

Cent ral fat igue may also be a fact or

• Polio virus infection of the motor strip & thereticular activating system

• Definition: "Increased mental effort

necessary t o per form a f ixed amount of muscle cont ract ion“

• PPS patients: feelings of fatigue (many report

hitting a "post polio wall")

Pulmonary Dysfunct ion



• Weakness of the breathing muscles (i.e. diaphragm &ribcage

• May cause: dyspnea on exertion (& at rest), poorclearance of respiratory secretions → ↑↑ risk of pneumonia, & ↑↑ resting arterial CO2 level

• Pulmonary function tests: restrictive pattern (small lungvolumes) → neuromuscular weakness based

• Mechanical ventilation may be required (very severecases)

⇒ small mechanical ventilators → deliver breathsthrough a comfortable plastic nose mask (performedwhile the patient is asleep at night ⇒ improved daytimefunction)

Sleep Disorders



• ↑↑ incidence of sleep disturbances + poor sleepquality & frequent awakenings

• Most important etiologies must be ruled out:central, obstructive & mixed sleep apneas

• Nocturnal hypoxemia & hypercarbia ⇒⇒

worsen the daytime function of the breathingmuscles

• Management: nocturnal non invasive ventilation

→ improve sleep quality & ↓↓ symptoms of daytime sleepiness (may also improve daytimerespiratory muscle function)

Dysphagia



• Eating / swallowing disturbances: more common inbulbar polio patients

• Evaluation: video fluoroscopy (pharyngeal const r ict or weakness )

• Laryngeal penetration & loss of the cough reflex: maybe asymptomatic ⇒

an underestimation of the

presence & severity of dysphagia (?)

• Patients may have already employed compensation(e.g. altering diet, cutting solids into small pieces,

chewing it thoroughly, taking small sips of liquids,eating slowly, & using postural maneuvers)

• Frequent accompanying conditions: progressive speechdifficulty (e.g. ↑↑ hoarseness, weakness, slurring)

Cold int olerance



• Occurs in ± 29% cases

• Limbs may be cold ⇒ cold exposureproduces weakness

• Involvement in the intermediolateral

column (?) ⇒⇒ vasoparesis, venous

pooling, & excessive heat loss

PAST HISTORY OF PPS



• Average age of polio onset: 7 yr

• Median time to max. recovery: 8 yr

• Median period of stable neurologic & functionalstatus: 25 yr

• Median post polio symptom duration before patient

presents for evaluation: 5 yr• Variables associated with shorter interval to PPS:greater severity & greater age.

• Initial symptoms: most freq. in LE →

affected in

acute illness ⇒ (UE weakness is easier to compensatew/o resulting in overuse)

• Onset: usually insidious (frequently precipitated by

injury, illness, bed rest, or weight gain)

recognizing the impacts of poliomyelitis in children and planning the rehabilitation strategies

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