What is postpolio syndrome?
New neuromuscular symptoms that occur decades after recovery from the acute paralytic episode
Primary& secondary impairments of postpolio
Primary: Flaccid muscle paresis/ paralysis.
Excessive or limited ROM
Secondary: fatigue, deep muscle pain, new muscle weakness
Other problems: muscle cramps, cold intolerance, swallowing difficulties, sleep disorders
These new impairments occur 25 years f
Postpolio etiology
Unknown
-recovered neurons are subject to premature aging and failure- cuz of
physiological weakness of the neuron
Dysfunction at NM juction.. no transmission of nerve impulse causing fatigue and decreased endurance
Long term goal for post polio
Provide principles/ methods for self management .
Short term goals for post poilio
-Alleviate and prevent the cause of pain
-Decrease the abnormally high workload of muscles relative to their limited capacity
-minimize postural and gait deviations mechanically
-Maintain and increase function, safety and quality of life
Interventions for pain
Muscle pain: nonfatiguing functional activities, energy conservation techniques, more frequent rest periods
Joint pain: rest to injured part, mechanical postural corrections, cold pack 6-8x daily. orthotics
ROM- passive, high reps, pain free motion; as pa
What are life threatening occurrences of post polio
Hypoventilation, dysphagia, & CP insufficiency.
Teach glossopharyngeal breathing and Manual assisted coughing
What is the rule of thumb for interventions of post polio
Estimate the intensity that the patient can tolerate and then apply one half, reduce the estimated reps to one half, and double the time of estimated rest periods
What is polio?
highly infectious lower motor neuron disease caused by the polio virus
What is the pathogenesis of polio? How does recovery come about?
1) 2 motor neurons are from the same motor neuron pool
2) one motor unit is affected by the polio virus and the motor neuron is destroyed
3) denervation of that muscle occurs
4) the majority of recovery is due to collateral sprouting from other healthy mo
Who will post polio syndrome effect? How will it present?
affects polio survivors
development of new neuromuscular symptoms in muscle previously affected
When can post polio syndrome present? How common is it?
can occur decades after original illness, with an average interval of 25 years
will affect 25-40% of polio survivors
What are risk factors for post polio syndrome?
must have polio diagnosis
long term overuse of muscles, this is believed to "tire out" nerve with collateral sprouting
What is the difference between post polio syndrome and post polio muscular atrophy?
while the distintinction can be unclear, in general post polio muscular atrophy is progressive weakness in muscles NOT previously affected by polio
What is the etiology of post polio syndrome?
unknown
since the majority of polio recovery is due to collateral sprouting, the bottom line is loss of collateral innervation
What is the pathogenesis of post-polio syndrome?
ongoing denervation
the compensated innervation no longer maintains that muscle fiber innervation
because of this, the NS prunes back the axonal sprouts that no longer have metabolic activity to support, leading to new denervation
What are the three most common clinical manifestations of post polio syndrome? What are other symptoms that can occur?
most common are muscle weakness, pain, and fatigue
can also have overuse of UEs due to weakened LEs
What aspect of post polio syndrome treatment are PTAs going to be the most involved in?
lifestyle changes and activity modifications
What is the prognosis for individuals with post polio syndrome?
slow progressive disorder that is rarely life threatening
may have stable periods that last 3-10 years
What may be a priority prior to starting an exercise program?
when fatigue is significant, lifestyle changes to conserve energy may be a priority because we have to keep them functional
When may strengthening not be indicated for individuals with PPS?
when weakness is significant, strengthening may not be recommended as it can further damage affected muscles
are stretching and aerobic exercise good for individuals with post polio syndrome?
yes, stretching and aerobic exercise should be considered whenever possible
What are precautions and contraindications for PT for individuals with PPS?
exercise should not cause muscle soreness or pain
exercise should not lead to fatigue that prevents participation in other activities that day or the days following
progression of exercise is slow, especially in those muscles that have not been exercised
What are the only muscles that you should strength train in an individual with PPS?
muscles that move through their full ROM
grade 3 MMT
What kind of exercise intensity is generally recommended for individuals with PPS?
minimal to moderate intensity exercise
What should aerobic exercise not cause in individuals with PPS?
muscle fatigue, generalized fatigue, muscle soreness, or pain
Bulbar Weakness (what CN and what are the s/s)
Myasthenia Gravis
-Impairment of CN IX-XII
-Dysphagia (drooling), Dysarthria (nasal twang), fatigue with chewing
Myasthenic Crisis
acute respiratory insufficiency due to weakness of diaphragmatic & intercostal muscles
suspect when s/s are worsening or started on new medication (prednisone)
Pathophysiology of MG
Autoantibodies against the ach receptor of the motor endplate that block & destroy acetylcholine receptors
Deep folds of the motor endplate are flattened and gap is widened
MG is a disorder where
motor end plate and neuromuscular junction
MG is a disorder of
neuromuscular transmission
MG has fluctuating
muscle weakness and fatigue
MG can happen at what age
any
T or F: MG is an autoimmune ds
true
What organ disorder raises the risk for MG
thymus
MG is associated with what type of conditions
autoimmune
What is decreased and changed/altered at the motor end plate
number of Ach receptors
What are cardinal signs and symptoms of MG
- skeletal muscle weakness
- fatigue
- no sensory involvement
- tendon reflexes are intact
There is no sensory or motor involvement?
sensory
Repetition with MG causes
fatigue
Immunologic testing shows what in the serum
Anti-Ach receptor antibodies
MG can fluctuate in intensity when
during the day
What is a medical emergency associated with MG
myasthenia crisis
What are the S/S of MG
Progressive muscle weakness; diplopia,ptosis;facial muscle weakness;dysphonia; only affects motor- not sensation or coordination
Name 6 Interventions for MG
1. Patient and family education. 2. Medication education and management. 3. Strategies to help with ocular manifestations. 4. Energy conservation. 5. Decrease risks of aspiration. 6. Avoid stress, infections, vigorous exercise, excessive environmental tem
What are the S/S of Myasthenic Crisis?
Respiratory distress; Increased RR, HR, BP; decrease urine output; dysphagia; dysarthria; ptosis,diplopia; prominent muscle weakness
What is Guillan Barre syndrome?
when the myelin sheath of peripheral nerves gets destroyed, preventing nerves from transmitting signals to the brain
What causes GBS?
unknown - our immune systems overreacts to some kind of stimulus that causes it to attack our LMNs at our peripheral nerves, causing inflammation
What is the main result of GBS?
LMN lesion
GBS causes LMN lesions. What would be seen with muscle tone and reflexes?
decreased muscle tone (flaccidity) and impaired reflexes (hyporeflexia)
Does GBS occur in the CNS or mostly in the periphery (PNS)?
no CNS involvement at all - only at the periphery
What is typically attacked first in GBS?
the myelin sheaths of peripheral nerves, causing disruption of conduction of messages trying to be sent
What is a bigger issue in GBS after the myelin sheath is destroyed?
the axon itself can be damaged, leading to poorer outcomes
Patients with GBS experience changes in what 2 things?
1. changes in sensory perception
2. changes in motor output
How does GBS progress?
it ascends from the feet and from the fingers (starts at the greatest periphery)
What periods of time in life is GBS more severe?
childhood GBS is less severe than adult GBS - most children GBS has 100% recovery
What is the incidence of GBS?
1-2 per 100,000 people
What are the 3 types of GBS?
1. acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
2. miller fisher syndrome (MFS)
3. acute motor axonal neuropathy (AMAN)/acute motor-sensory axonal neuropathy (AMSAN)
In GBS, weakness progresses in what direction?
caudal to cephalal (distal to head lastly)
Patients with GBS can complain of what abnormal sensations?
numbness and tingling which can last for a long time
Does weakness in GBS progress rapidly?
yes, quite rapidly from the feet upwards
What happens to reflexes in GBS?
decreased or absent
What occurs to vibration, proprioception, and touch abilities in GBS?
lost
What occurs to breathing and HR in GBS?
decreased
What occurs to a patient's level of consciousness in GBS?
severe fatigue
What occurs to a patient's cranial nerves?
they can be involved as the disease progresses
Can GBS cause autonomic dysfunction meaning they stop breathing on their own and thinking on their own?
yes
Does GBS progress symmetrically from L to R?
yes
How long can it take for the disease to progress?
2 days to 4 months
How long can it take for someone with GBS to have full recovery and be healthy again?
4 months
Because the treatment progresses distal to proximal, recovery occurs in what order?
proximal to distal (probably because peripherally, the damage has been occurring for longer duration than it has centrally)
About 30% of people who had GBS, still continue to have what up to 2 years after the onset of their disease?
1. motor weakness
2. numbness and tingling
When do most patients ambulate after getting GBS?
in about 6 months post symptom onset
When should treatment be given to those with GBS in order for that patient to have the best chance of recovery?
within the first 2 weeks of onset
What is provided in the mattress for GBS patients?
a low pressure mattress
What is given to GBS patients to prevent contractures?
multi-podus boots and PROM
What can PTAs do for patients with GBS?
provide the low pressure mattress to avoid skin breakdown, provide TEDS or intermittent compression (plastic socks that inflate and deflate to act as artificial muscle pump) to prevent phlebitis, provide multi-podus boots to remove heel pressure from bed
What should patients with GBS consider for therapy?
aquatic therapy
What in exercise is essential for GBS patients?
regular exercise is essential - 8-10 short bouts daily progressing to longer bouts as tolerated
Does PTA accelerate nerve healing in GBS patients?
no, exhaustion can cause injury, do not exercise the patient into fatigue
Why does aquatic therapy help patients with GBS so much?
because gravity acts like a resistive barrier, and water unweights them and provides a compressive force which helps give the brain information about where the body is in space
In GBS patients, because these patients are younger and have never dealt with this severe of an illness, do they often push limitations?
yes
What is the best types of PTA training to provide for your patients with GBS according to the ADIP article?
practicing motor skills for neurodevelopmental positions (from supine to sit to stand) with ROM and strengthening at each stage - high intensity group does better overall with chronic GBS - bike training group does well
Most Common GB
-acute inflammatory demyelinating polyneuropathy(AIDP)
GB
-demyelinating disease of PNS
-reaction of bodys immune system to PNS
-attack against schwann cells
-macrophages and lymphocytes migrate to nodes of ranvier
Guillain Barre
A nervous system disorder in which the immune system attacks the myelin sheaths of the peripheral nerves
What causes demyelination?
Macrophages and lymphocyte migration to the nodes of Ranvier and the attack the Schwann cells.
What does demyelination of a nerve cause?
degradation of nerve impulses or complete loss
What is the presentation of GBS
Motor and sensory interruption
Tingling in legs
rapidly evolving symmetrical
ascending weakness or flaccid paralysis
pain
What occurs in the recovery phase of GBS
Re-myelination occurs and some sort of restoration of sensory and motor function
How is GBS diagnosed
Subjective history
clinical presentation
nerve conduction velocity
spinal tap
What is the prognosis of GBS?
Early diagnosis equals better prognosis
6 mo 80% people will be walking
20% will have lasting severe disability
Amyotrophic Lateral Sclerosis (ALS)
Rapidly progressive and fatal neurodegenerative disorder of motor neurons; affects both corticospinal and bulbar/spinal motor neurons; characterized by scarring in lateral tracts of spinal cord and deposition of cytoplasmic protein inclusions
Symptoms of ALS
Progressive muscle weakness, subtle cognitive changes
what facilitates the transport of signals along a nerve fiber
myelin sheath
do MS symptoms have gradual or rapid onset?
gradual
MS has periods of __ and __
remission
exacerbation
with MS periods of remission, what may still be noticeable?
impairment
life expectancy after onset of symptoms:
25 years
what is usually the cause of death in MS?
infection
(D/T immobility)
etiology of MS:
unknown
what process involves:
antigens activating T cells (lymphocytes) that then migrate to the CNS form systemic circulation to disrupt the BBB
autoimmune process
inhibit relapsing-remitting MS that suddenly begins to have a decline without periods of remission
secondary progressive MS
steady increase in disability without attacks
primary progressive MS
unpredictable attacks which may or may not leave permanent deficits followed y periods of remission
relapsing-remitting MS
because MS affects the nerve impulses to the pupils, what is a clinical manifestation?
double/blurred vision
MS may cause optic neuritis which will cause:
blindness
(in one eye, temporary)
MS may cause LE or hand numbness/tingling/prickling which is
paresthesia
__ is aggravated by heat & humidity, deconditioning, & medication SE
fatigue
what will be seen on an MRI in a person who has MS but is not a definitive diagnosis?
sclerotic plaques
Multiple Sclerosis - Patient Education
Good balance of exercise and rest to prevent fatigue
Avoiding or decreasing precipitating factors.
Building resistance to illness.
avoid exposure to cold climates
Avoid exposure to people who are sick
vigorous and early treatment of infection when it does
relapsing remitting
most common type of MS
secondary progressive
cessation of fluctuations with slow deterioration
MS diagnosis
Two or more neurologic events separated in time or a progressive course over 1 year, with objective demonstration of two or more affected areas of the CNS
multiple sclerosis
Autoimmune disease of the CNS (spinal cord, brain, optic nerve). Inflammatory disease of the white matter tissue of the brain
Relapsing/Remitting (85%)
follows a pattern of unpredictable, recurrent, attacks followed by gradual recovery, generally over weeks or months. Any impairment will not worsen between attacks
Primary Progressive (10%)
Characterized by a gradual progression of the disease from its onset with no remissions at all
Secondary Progressive MS
Slow and steady disease progression that transitioned from the relapsing-remitting type.
unpredictable
Symptoms of MS are ___________ and vary from person to person.
Most common s/s of MS
Bladder/Bowel dysfunction, changes in Cognitive Functioning, Dizziness and Vertigo (perception of motion), Emotional problems, Sexual dysfunction, Visual problems, spasticity/weakness, pain
Less common s/s of MS
Hearing loss, speech/swallowing dysfunction, itching, seizures, headaches
MS on exercise
lower ex. tolerance and higher perceived fatigue, low max aerobic capacity and peak aerobic capacity, blunter HR and BP responses, decrease in muscle performance, lower isometric and dynamic power production
True
with MS the body attacks its own tissue (myelin) and scientists do not yet know what triggers the immune system to do this?
role of environment on MS
Whites in US temperate climates, disease prevalance increases with distance from equator, "epidemics" people living off the coast of Denmark post WW2, environmental causes and viruses (mononucleosis, varicella-zoster/ chicken pox and shingles, hepatitis v
systemic fatigue
this is common in MS but tends to improve with increased physical fitness.
general centrally mediated MS fatigue and temporary peripheral exercise related fatigue
it is important to help the individual with MS distinguish between what?