Post-Poliomyelitis Syndrome (PPS)
This leads to distal
Definition
- It is a disorder typically characterized by the
gradual onset and slow progression of new
weakness, fatigue, and pain in survivors of
paralytic poliomyelitis after a period of
partial or complete recovery and many
years of neurologic stability.
- The symptoms are persistent and cannot be
explained by the presence of other
neurologic, medical, or orthopedic
conditions
Other Names
 Post-polio syndrome
 Progressive post-poliomyelitis muscular
atrophy
EPIDEMIOLOGY
Gender
- Female > Male (Harrison’s Internal Medicine)
- 
Incidence
- Estimates vary from 22% to 85% of individuals
who survive acute poliomyelitis
Peak Incidence
- About 30 to 36 years following acute
poliomyelitis, although cases have been
reported between 8 and 71 years.
Risk Factors
- Increasing age and greater length of time
since the episode of acute poliomyelitis
- Acute poliomyelitis at an older age
- Greater severity of the acute poliomyelitis or
chronic deficits after recovery
Pathology
- Loss of the anterior horn cell
Etiology
- unknown; possible hyperfunctioning of
motor neurons, long-term overuse at high
levels resulting in new denervation
(Siegelman)
- The most popular hypothesis is
that the reduced population of enlarged
motor units after acute poliomyelitis is
subject to increased stress and overuse over
the course of years.
degeneration of the motor units and
worsening denervation, causing clinical
symptoms.
- Peripheral disintegration model Commented [JPE1]: Most widely held theory for PPS
- Over-sprouting of new axon terminals from surviving
Physical Findings & Clinical Presentation LMNs occur in the immediate
aftermath of the acute paralytic poliomyelitis
 New weakness and atrophy, asymmetrical, - This compensatory distal reinnervation expands the
proximal and slowly progressive in size of motor units and provides effective motor function;
distribution. Occurs in both initially weak and this stabilizes muscle strength for many years.
- However, this extensive nerve sprouting also increases
uninvolved muscles.
the metabolic burden of surviving LMNs, so after many
 Abnormal fatigue: may not be related to years, an un-identified process 1st causes nerve terminal
activity levels, doesn't recover easily with dysfunction presenting as fatigue and then nerve
usual rest periods. terminal disintegration presenting as muscle weakness
and atrophy.
 Pain: myalgia, cramping pain, joint pain with
repetitive injury, hypersensitivities
 Decreased function with reduced Commented [JPE2]: Hitting a wall phenomenon/Polio
wall: lack of energy with minimal activity; occurs primarily in
endurance for routine activities.
the afternoon
 Slow progression, either steady or step-wise Most disabling accompaniment and most debilitating
 Environmental cold intolerance symptoms; may affect patient’s cognition & thinking
 Brain fatigue-generator model Fatigue occurs commonly in late afternoon/early evening
Atypical Fatigue: Fatigue that last throughout the day
 Sleep disturbances & weakness in respiratory
system
Depression Commented [JPE3]: Joint pain may d/t wear & tear on
 Neurologic examination reveals evidence of joints, poor posture & deterioration of soft-tissue or
surgeries done to treat the residual effects of polio
asymmetric, lower motor neuron weakness
with or without musculoskeletal deformities Commented [JPE4]: More than 65% of individuals with
PPS have reported
secondary to longstanding polio such as
neck, shoulder, and back pain radiating to the hip and leg
asymmetric limb size, kyphoscoliosis, (Umphred)
degenerative joint disease, and joint
Commented [JPE5]: Difficulty in concentration, memory
instability d/t the use of assistive devices and
attention; damage in reticular formation, hypothalamus,
wheelchairs for decades dopaminergic neurons
 Postural abnormalities
Commented [JPE6]: Nocturnal hypoventilation occurs
Less Common Presentation less frequently
Commented [JPE7]: -Forward head, forward-leaning
 Fasciculations
trunk, (-) lumbar curve, uneven pelvis, scoliosis, lateral trunk
 Dysphagia shifts
 Dysarthria
Criteria for Diagnosing Post-Poliomyelitis Syndrome
- AKA: Halstead-Ross Criteria
- Hx of polio
- recovery period & neurological stability at
least 15 years
- Onset of >2 S/Sx:
 fatigue,
 mm/joint pain
 weakness & atrophy in new mm,
 functional loss
 cold intolerance
- persistent S/Sx for 1 yr.
- Rule out other diagnosis

Ancillary Procedure
1. Nerve Conduction Velocity (NCV)
- SNAP: Normal
- CMAP: Abnormal (Low maximum CMAP
amplitudes)
2. EMG
- Abnormal activity
- Delayed Recruitment
- (+)Giant motor neuron unit, may indicative
of chronic denervation and reinnervation
- Fasciculations can be unusually coarse and
large in keeping with the giant motor units
detectable
3. Low Rate Repetitive Stimulation (LRRS)
- Normal activity
4. SFEMG (Single Fiber EMG)
- Increased jitter, fiber density and blocking
5. Muscle Biopsy
- usually shows acute and chronic
neurogenic atrophy and often marked
group muscle fiber atrophy and fiber type
grouping
Lab Exam
1. CBC, Electrolytes, Thyroid Function Test
- To rule out other cause of fatigue
- Typically normal
2. Erythrocyte Sedimentation Rate (ESR)
- Normal
- To rule out auto-immune disorders
- Increase Creatine-Kinase
Examination
1. History
- confirm original acute polio illness;
document onset of present symptoms,
presentation, course, chronology.
2. Motor: strength, atrophy, muscle
fatigue, muscle twitching and cramps
- strength, atrophy, muscle fatigue, muscle
twitching and cramps
- Identify functional contractions (fair grades
or above)
- Look for spotty involvement, asymmetrical
paralysis
3. ROM & Deformity
4. Pain
- Muscle pain: check tenderness to touch
- Skeletal, soft tissue pain: chronic overuse or
poor alignment
5. Sensory function
- any sensory deficit is due to other etiology
(sensation is unaffected in PPS).
6. Respiratory Function
- Examine for dyspnea, difficulty in speaking
and weak cough
7. Aerobic Capacity
- use ergometer that involves both upper and
lower extremities; e.g., Schwinn Air-Dyne,
discontinuous protocoL submaximal test
(ACSM recommendation)
Drugs
- There are no specific drugs to treat Post-
polio syndrome
a. Anti-depressants
- e.g., amitriptyline (Elavil), fluoxetine (Prozac)
b. Neurotransmitters inhibitors
- decreases fatigue and sleep disorders; e.g.,
serotonin, norepinephrine
PT GOALS, OUTCOMES
1. Maintain respiratory function
- Teach breathing exercises, supportive
cough maneuvers, postural drainage as
indicated
2. Teach energy conservation techniques,
activity pacing: balance activity with
frequent rest periods to decrease fatigue,
prevent overwork damage in weakened,
denervated muscle.
- Teach relaxation techniques to maximize
rest
- Avoid unnecessary activities to maximize
important work.
3. Preserve or increase muscle strength
- Provide moderate exercise program (non-
exhaustive exercise): modified strengthening
and conditioning; use low-intensity,
discontinuous non-fatiguing exercise with
increased rest periods.
- Caution against widespread use of strength
training, it may trigger fatigue
- Consider pool programs: minimizes overwork Commented [JPE8]: Aquatic exercise can be very
relieves pain; general body conditioning beneficial because water decreases the stress on the joints,
4. Aerobic Conditioning bones, and muscles.
- moderate to low-level training depending
upon class of disease, discontinuous
protocol. In severe atrophic polio, exercise is
contraindicated
5. Maintain or increase function
- provide recommendations for lifestyle
modification; minimize abnormal postures,
gait deviations.
6. Prescribe appropriate orthoses, mobility aids Commented [JPE9]: The most frequently prescribed
(motorized cart), assistive devices, orthoses include
environmental modifications shoe lifts, AFOs (rocker bottom) and KAFOs.
7. Eliminate or control pain: provide options for
pain controL foster self-control
8. Teach patient and family all care, activities
of daily living; lifestyle modifications
9. Provide psychological support and
reassurance