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Post Polio Syndrome (PPS)

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The key takeaways are that poliomyelitis is a viral disease that can affect nerves and lead to paralysis. It is caused by the poliovirus which spreads through direct or indirect contact with infected individuals or their feces. The virus destroys motor neurons leading to muscle weakness and paralysis.

The main symptoms of poliomyelitis include fever, headache, stiffness in the back and neck, asymmetrical muscle weakness, sensitivity to touch, difficulty swallowing, muscle pain, and loss of reflexes. Paralysis is most common in the legs but can also affect other areas.

The main types of polio are spinal polio, bulbar polio, and paralytic polio. Spinal polio affects the spinal cord and causes leg paralysis. Bulbar polio affects the brainstem and causes difficulties swallowing and breathing. Paralytic polio causes the most extensive muscle weakness and paralysis.

Poliomyelitis

Poliomyelitis is a viral disease that can affect nerves and can


lead to partial or fullparalysis.
Poliomyelitis is a disease caused by infection with the
poliovirus.

The virus spreads by:


Direct person-to-person contact.
Contact with infected mucus or phlegm from the nose or
mouth.
Contact with infected feces.

Risks include:
Lack of immunization against polio.
Travel to an area that has experienced a polio outbreak.
Paralytic polio
Poliovirus spreads along certain nerve fiber
pathways, preferentially replicating in and
destroyingmotor neuronswithin thespinal
cord,brain stem, ormotor cortex.
The destruction of neuronal cells
produceslesionswithin thespinal ganglia.
Inflammation associated withnerve cell
destruction often alters the color and appearance
of the gray matter in thespinal column, causing it
to appear reddish and swollen.
Other destructive changes associated with
paralytic disease occur in theforebrain region,
specifically thehypothalamusandthalamus.
Early symptoms of paralytic polio include:
high fever
headache
stiffness in the back and neck
asymmetrical weakness of various muscles
sensitivity to touch
difficulty swallowing
muscle pain
loss of superficial and deepreflexes
constipation, or difficulty urinating
paralysis of one leg is most common in adults,
extensive paralysis of thechestandabdomenalso
affecting all four limbs(quadriplegia)
Spinal polio

Spinal polio, the most common form of paralytic


poliomyelitis, results from viral invasion of the motor
neurons of theanterior horn cells, or theventral
(front)grey mattersection in thespinal column, which
are responsible for movement of the muscles (include
trunk,limbs, and theintercostal muscles).
Virus invasion causes inflammation of the nerve cells,
leading to damage or destruction of motor
neuronganglia. When spinal neurons die,Wallerian
degenerationtakes place, leading to weakness of those
muscles formerly innervatedby the now-dead neurons.
With the destruction of nerve cells, the muscles no
longer receive signals from the brain or spinal cord.
Without nerve stimulation, the musclesatrophy,
becoming weak, floppy and poorly controlled, and
finally completely paralyzed.
Symptoms include:
Fever and muscle pain.
Deeptendonreflexesaffected (absent or
diminished)
The virus may affect muscles on both sides
of the body, but more often the paralysis
isasymmetrical.
Anylimb or combination of limbs may be
affected - one leg, one arm, or both legs and
both arms.
Paralysis is often more
severeproximally(where the limb joins the
body)than distally (fingertips and toes).
Bulbar polio

Bulbar polio occurs when poliovirus invades and


destroys nerves within thebulbarregion of thebrain
stem.The bulbar region is awhite matterpathway
that connects thecerebral cortexto the brain stem.
The destruction of these nerves weakens the
muscles supplied by thecranial nerves, producing
symptoms ofencephalitis, and causesdifficulty
breathing, speaking and swallowing.
Critical nerves affected are theglossopharyngeal
nerve thevagus nerve, and theaccessory nerve.
Due to the effect on swallowing, secretions
ofmucusmay build up in the airway, causing
suffocation.
Other sign and symptoms:
Facial weakness
Double vision
Difficulty in chewing
Abnormalrespiratory rate, depth,
and rhythm
Pulmonary edema
Shockmay lead to fatal
Bulbospinal polio

Approximately 19% of all paralytic polio cases have


both bulbar and spinal symptoms this subtype is
called respiratory or bulbospinal polio.
Here, the virus affects the upper part of the cervical
spinal cord (C3 through C5), and paralysis of the
diaphragmoccurs.
The critical nerves affected are thephrenic nerveand
those that drive the muscles needed for swallowing.
By destroying these nerves, this form of polio affects
breathing, making it difficult or impossible for the
patient to breathe without the support of aventilator.
It can lead to paralysis of the arms and legs and may
also affect swallowing and heart functions.
Postpolio syndrome (PPS) is a neurologic
disorder characterized by new and
progressive muscular weakness, pain, and
fatigue many years after the acute paralytic
polio.

Halstead introduced the term "post-polio


syndrome" in 1986, and he published
revised criteria for diagnosing PPS in 1991,
in which new muscle weakness was
introduced as an obligatory criterion.
(Halstead LS.
1991 )
Criteria for diagnosed of PPS
Prior paralytic poliomyelitis with evidence of motor
neuron loss, as confirmed by history of the acute
paralytic illness, signs of residual weakness and atrophy
of muscles on neurologic examination, and signs of
denervation electromyography (EMG)
A period of partial or complete functional recovery after
acute paralytic poliomyelitis, followed by an interval
(usually 15 y or more) of stable neurologic function
Gradual or sudden onset of progressive and persistent
new muscle weakness or abnormal muscle fatigability
(decreased endurance), with or without generalized
fatigue, muscle atrophy, or muscle or joint pain; sudden
onset may follow a period of inactivity, or trauma or
surgery; less commonly, symptoms attributed to PPS
include new problems with breathing or swallowing.
Symptoms persist for at least a year
Exclusion of other neurologic, medical, and orthopedic
problems as causes of symptoms
pathophysiology
decompensation of a chronic denervation and
reinnervation process to the extent that the remaining
healthy motor neurons can no longer maintain new
sprouts; thus, denervation exceeds reinnervation.
(Gonzalez H, Ottervald J, Nilsson KC, et al.
2009)

motor neuronal loss due to reactivation of a persistent


latent virus.

Infection of the polio survivor's motor neurons by an


enterovirus
Signs & Symptoms
1) Fatigue
general fatigue
physical fatigue
mental fatigue
modifiable factors such as stress, depression, pain,
and physical activity account for a portion of fatigue
in PPS.

2) Muscle weakness
In PPS, muscle weakness and atrophy can be
permanent, because of loss of motor units, or
transient, because of muscle fatigue.
3) Muscle pain
Deep aching pain may be a
component of a myofascial pain
syndrome or fibromyalgia.

Pain symptoms were reported by 57


(90.5%) of the 63 study participants; pain
was reported most often in the shoulders,
legs, hips, and lower back, with the most
intense pain occurring in the legs, knees,
wrists, head, and lower back.
4) Gait disturbance
Difficulty with gait is caused by progressive
weakness, pain, osteoarthritis, or joint instability

5) Respiratory problems
residual respiratory muscle weakness.
These changes cause chronic microatelectasis,
diminished pulmonary compliance, increased chest
wall tightness, chronic alveolar hypoventilation,
decreased cough and expiratory flow, and
decreased clearing of secretions.
The new respiratory difficulties are not only related
to new respiratory muscle weakness but also to
scoliosis, pulmonary emphysema, cardiovascular
insufficiency, or poor posture.
6) Swallowing problems (dysphagia)
Dysphagia can occur in patients with
bulbar and nonbulbar postpolio.

Subclinical asymmetrical weakness


in the pharyngeal constrictor
muscles is almost always present in
all postpolio muscular atrophy
patients.
7) Sleep apnea
This disorder is not uncommon in patients left with
residual bulbar dysfunction or severe respiratory
compromise. Patients with bulbar involvement had
more frequent sleep apnea than patients without.

Sleep apnea appears to be due to a combination of


the following:

Central apnea, due to a residual dysfunction of the


surviving bulbar reticular neurons
Obstructive apnea, due to pharyngeal weakness
and increased musculoskeletal deformities from
scoliosis or emphysema
8) Flat-back syndrome
the inability to stand erect because of forward
flexion of the trunk and pain in the low back and
legs.

The flat-back syndrome typically occurs in


patients with diminished lumbar lordosis as a
result of instrumentation of the spine for scoliosis,
vertebral fracture, or degenerative joint disease.

The trunk extensor musculature plays an essential role in


maintaining upright posture, and it may be that PPS-related
weakness in this musculature represents a major contributing
factor to the flat-back syndrome in these patients
Muscle dystrophy
Beckers Muscular Dystrophy
Becker muscular dystrophy (BMD)is one
of nine types of muscular dystrophy, a
group of genetic, degenerative diseases
primarily affecting voluntary muscles.
BMD is similar to DMD but allows the
voluntary muscles to function better than
they do in DMD.
The heart muscle, however, can be
affected similarly to the way it is in DMD.
BMD occurs when the dystrophin protein
that's made from a particular gene on the
X chromosome is only partially functional.
While DMD mutations cause virtually no
functional dystrophin to be made, people
with BMD make dystrophin that is partially
functional.
They make a shortened form of the
protein, which protects the muscles of
those with Becker from degenerating as
completely or as quickly as those of
people with DMD.
Symptoms
BMD's onset is usually in late childhood or
adolescence, and the course is slower and
less predictable than that of DMD.
Generalized weakness first affects muscles
of the hips, pelvic area, thighs and
shoulders.
Calves are often enlarged.
Duchenne Muscular
Dystrophy
Lack of the protein dystrophin causes
muscles to deteriorate and break
down progressive difficulty with
walking and general mobility.
One of the most rapidly progressive
of the childhood neuromuscular
disorders.
Mostly boys are affected.
Symptoms
A delayed walking age with first steps being taken at around
eighteen months.
Has difficulty climbing (stairs for example), difficulty running
and jumping, and he may develop a waddling gait.
Symptoms include enlargement (hypertrophy) of the calf
muscles, in about 90% of cases.
He may develop a tendency to walk on his toes and this is
often accompanied by a protruding abdomen and sway-backed
stance, called lordosis.
GOWERS SIGN- difficulty getting up from the floor without
help, he may use his hands to climb up his legs for support .

These symptoms generally begin to develop between the ages of one


and three years and continue to progress until he requires wheelchair
support, most often between the ages of eight and twelve.
Other problems related to
MD
1) Respiratory function
2) Heart problems
3) Learning difficulties
4) Pain and sensation
Physical treatment
exercise
General principle of exercise for polio survivor:
The intensity of exercise is low to moderate
The progression of the exercise is slow,particularly
in muscle that not have been exercised for a
period of time or/and have a obvious chronic
weakness from acute poliomyelitis.
Pacing is incoperate into the detailed program.
The plan should include a rotation of exercise
types, such as strecthing, general(aerobic)
conditioning, strengthening endurance, or joint
range of motion exercise.
Exercise prescription should include:
1) The specific muscle group to be included
2) The specific muscle group should be excluded
3) The type of exercise together with frequency
and duration

Exercise should be reduce discontinued if cause


4) Additional weakness
5) Excessive fatique

No muscle should be exercise to the point of causing


ache,fatigue, and weakness
Physical treatment
exercise :- pt not push to the point of
exhaustion
:- too much exs & wrong type exs
additional muscle damage
Eg : passive strecthing / assisted strecthing
: hydrotherapy / swimming
Cognitive behaviour therapy (reduce
fatigue)
Electrical stimulation, biofeedback,
muscle relaxation exs
Gait training
-supportive equipment
a)splints: maintain jt ROM, prevent
contracture

-medical
Steroid(glucocorticoid,corticosteroid)

-surgery
Release contracture

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