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NMR II > Final: ALS and Post-Polio Syndrome > Flashcards

Final: ALS and Post-Polio Syndrome Flashcards

1
Q

What does ALS stand for?

A

Amyotrophic Lateral Sclerosis

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2
Q

What does amyotrophy mean?

A

Muscle atrophy

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3
Q

What does lateral relate to with ALS?

A

Involvement of the lateral and anterior corticospinal tracts

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4
Q

What does sclerosis relate to with ALS?

A

Glial cell proliferation and hardening

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5
Q

What nerves does ALS affect?

A

UMN, LMN, CN

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6
Q

What is ALS?

A

Progressive neurodegenerative motor neuron disease that affects the motor neurons in the spinal cord, brainstem, and brain

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7
Q

What is the etiology of ALS?

A

90% unknown, 10% familial

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8
Q

What are possible causes of ALS?

A

Excitotoxicity caused my excess glutamate and oxidative damage from free radicals

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9
Q

Destruction of what three structures in the cerebral cortex lead to UMN signs?

A
  1. Pyramidal cells
  2. Corticospinal tract
  3. Corticobulbar tract
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10
Q

Destruction of what type of neurons lead to LMN signs?

A

Alpha motor neurons

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11
Q

Destruction of what two structures in the cerebral cortex lead to LMN signs?

A
  1. Anterior horn of spinal cord
  2. Cranial nerve nuclei in brainstem
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12
Q

What are five other possible areas that can be affected by neuronal loss?

A
  1. Frontotemporal cortex
  2. Thalamus
  3. Basal ganglia and SN
  4. Spinocerebellar tracts
  5. Dorsal columns
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13
Q

What are examples of LMN signs?

A

Asymmetric, distal weakness, extensors weaker than flexors, cervical extensor weakness, bulbar signs (CN IX_XII), hyporeflexia, hypotonicity, atrophy, muscle cramps, fasciculations

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14
Q

What are examples of UMN signs?

A

Spasticity, hyperreflexia, clonus, pathological reflexes, muscle weakness, pseudobulbar palsy due to corticobulbar tract

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15
Q

What will happen to UMN symptoms over the course of the disease?

A

They can decrease

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16
Q

Where will pt’s with ALS typically have weakness?

A

Extensors of UE and flexors of LE

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17
Q

What is the presentation of pseudobulbar palsy?

A

Spastic - UMN of the corticobulbar tract

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18
Q

What is the presentation of bulbar palsy?

A

Flaccid - LMN

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19
Q

What is the clinical presentation of bulbar symptoms?

A

Dysarthria, dysphagia, sialorrhea, pseudobulbar affect

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20
Q

What CNs are affected and not affected by ALS?

A

Affected: V, VII, IX, X, XII
Non affected: ocular muscles

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21
Q

What is preserved with ALS?

A
  1. Eye movement
  2. Bowel and bladder
  3. Sensory system
  4. Cognition 50%
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22
Q

What are three primary early signs of ALS?

A
  1. Asymmetrical weakness of distal aspect of one limb
  2. Cramping with volitional movement, early morning stiffness
  3. Muscle fasiculations, spontaneous twitching of muscle fibers
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23
Q

What are two major later manifestations of ALS?

A
  1. Respiratory complications
  2. Oral motor complications
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24
Q

What is required for an ALS diagnosis?

A

Clinical presentation of both UMN and LMN symptoms, muscle biopsy with denervation atrophy, muscle enzymes, normal CSF, no changes to myelogram

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25
Q

What is the first FDA approved drug for ALS?

A

Riluzole

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26
Q

What is Riluzole, and what are the effects?

A

Inhibits glutamate. May have neuroprotective effect. Slows the progression by 10-15% and increased survival by 3 months

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27
Q

What is Radicava, and what are the effects?

A

Targets oxidative stress. Still ongoing

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28
Q

What types of medications are used to control drooling with ALS?

A

Anticholinergics

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29
Q

Onset before what age can cause a slower progression of ALS?

A

50

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30
Q

What is the typical survival duration after symptom onset of ALS?

A

2-5 years

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31
Q

Does bulbar or limb onset lead to a more rapid progression?

A

Bulbar onset

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32
Q

Practice Question: What is the average survival after symptom onset of ALS?

  1. 1-2 years
  2. 2-3 years
  3. 3-5 years
  4. 5-10 years
A
  1. 3-5 years
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33
Q

Practice Questions: Which of the following symptom presentation would be characteristic of someone with a possible dx of ALS?

  1. Muscle spasm and diaphoresis
  2. Foot drop and sialorrhea
  3. Fatigue and sudden vision loss
  4. Hearing loss and fasciculations
A
  1. Foot drop and sialorrhea
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34
Q

Practice Question: Which of the following systems tend to be preserved in people with ALS?

  1. Motor function
  2. Cranial nerve function
  3. Respiratory function
  4. Sensory function
A
  1. Sensory function
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35
Q

What are the three pharmacological agents for ALS?

A
  1. Rilutek
  2. Radicava
  3. Relyviro (AMX)
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36
Q

What is the MOA of Relyviro (AMX)?

A

Prevents motor neuron death by working at the endoplasmic reticulum and mitrochondria

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37
Q

What is the functional impact of Relyviro (AMX)?

A

Significantly reduce the rate of decline on the Revised ALS Functional Scale

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38
Q

What is the effect of Relyviro (AMX) on life expectancy?

A

Increased by 6.5-10 months - initial trials

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39
Q

What body function and structural impairments are relevant to examining with a pt with ALS?

A

ROM, MMT, tone, CN integrity, sensory function, cough effectiveness, chest expansion, respiratory sounds, integumentary

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40
Q

What activity limitations are relevant to examining with a pt with ALS?

A

Functional independence with mobility related tasks, including bed mobility, transfers, gait, balance

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41
Q

What participation restrictions are relevant to examining with a pt with ALS?

A

QOL measures, ALS Assessment Questionnaire, Fatigue Severity Scale

42
Q

What does it mean if a person with ALS scores below a 3/5 MMT?

A

You have to be cautious not to overwork their muscles because it can lead to further degeneration of muscle fibers

43
Q

What does it mean if a person with ALS scores above a 3/5 MMT?

A

Muscle cells can take more loading and still have the ability to hypertrophy

44
Q

What are the two outcomes measures that are specific to ALS?

A
  1. ALS Functional Rating Scale-Revised
  2. ALSAQ-40
45
Q

What 12 areas of function are included in the ALS Functional Rating Scale-Revised?

A
  1. Speech
  2. Salivation
  3. Swallowing
  4. Handwriting
  5. Cutting food and handling utensils
  6. Dressing and hygiene
  7. Turning in bed
  8. Walking
  9. Climbing Stairs
  10. Dyspnea
  11. Orthopenea
  12. Respiratory insufficiency
46
Q

What does a lower score on the ALS Functional Rating Scale-Revised indicate?

A

Lower level of functioning

47
Q

What are the 5 dimensions of health assessed in the ALSAQ-40?

A
  1. Mobility
  2. ADLs
  3. Eating
  4. Drinking
  5. Emotional Functioning
48
Q

What does a higher score on the ALSAQ-40 indicate?

A

Higher score indicated more severely impaired

49
Q

What are components of palliative care for ALS?

A

SLP consult, respiratory, pain, mental health, cognitive implications

50
Q

What is sialorrhea, and how is it treated?

A

Excessive saliva. Treated with anti-cholinergics to decrease production

51
Q

What is dysphagia?

A

Impaired chewing or swallowing

52
Q

What is dysarthria and what is typically affected?

A

Impaired speech. Effects voice projection and enunciation

53
Q

Why are respiratory impairments dangerous?

A

Increases risk of respiratory infections

54
Q

How frequently will a pt with ALS have a pulmonary function test?

A

Every three months

55
Q

What is recommended when vital capacity drops below 50%?

A

Non-invasive positive pressure ventilation

56
Q

Why are CPAP, BiPAP, and O2 not recommended for ALS?

A

Suppresses the respiratory drive

57
Q

When non-invasive positive pressure ventilation is not longer effective, what happens?

A

Invasive ventilation or hospice-care

58
Q

What are intervention strategies for muscle cramps and spasticity?

A

Hydration, nutrition, stretching

59
Q

What medical management is appropriate for muscle cramps and spasticity?

A

Cramps: anticonvulsants
Spasticity: benzodiazepines
Pain: NSAID, opioids
Fasiculations: ativan

60
Q

What are pharmacological interventions for anxiety and depression with people with ALS?

A

SSRIs, tricyclic antidepressants, benzodiazepines

61
Q

What is positive psychological well-being associated with in ALS?

A

Prolonged survival

62
Q

What % of pt’s with ALS show significant cognitive impairment?

A

35.6%

63
Q

What does ALS-FTD stand for?

A

ALS Frontotemporal Dementia

64
Q

What is ALS-FTD?

A

Cognitive decline, executive functioning impairments, difficulty with planning, organization, and concept abstraction. personality and behavior changes

65
Q

What cognitive impairments do pt’s with ALS who do not have ALS-FTD show?

A

Difficulties with verbal fluency, language comprehension, memory, abstract reasoning, generalized impairments in intellectual function

66
Q

What does ALS-CBS stand for?

A

ALS Cognitive Behavioral Screen

67
Q

What does the ALS-CBS assess?

A

Completed by a caregiver, assesses cognition and behavioral changes in order to determine if a pt has ALS-FTD

68
Q

Practice Question: Identify two strategies as a PT may suggest or incorporate in the POC to address muscle cramping?

A

Hydration and stretching

69
Q

Practice Question: Which of the following outcome measures is most commonly used in people with ALS to tract functional decline?

a. ALSFRS-Revised
b. ALS Cognitive Behavioral Screen
c. BERG
d. ALD-AQ 40

A

a. ALSFRS-Revised

70
Q

Practice Questions: Which of the following conditions seen in people with ALS warrant a referral for SLP? (select all that apply)

a. Sirrholea
b. Dysphagia
c. Motor deficits
d. Dysarthria

A

a. Sirrholea
b. Dysphagia
d. Dysarthria

71
Q

What are the goals of early stage rehab for ALS?

A

Preventative and restorative, introduce some compensatory techniques

72
Q

What are the goals of middle stage rehab for ALS?

A

Compensatory and preventative, less focus on restorative

73
Q

What are the goals of late stage rehab for ALS?

A

Compensatory and preventative, limited focus on restorative

74
Q

Describe motor neuron degeneration

A

In the early phase, reinnervation can compensate for loss of strength. Once 50% of motor units are lost, reinnervation can no longer compensate

75
Q

What are the 5 benefits of exercise with ALS?

A
  1. Stress reduction
  2. Maintaining strength as long as possible
  3. Staying flexible as the body becomes more sedentary
  4. Improving sleep
  5. Maximizing respiratory and cardiac function
76
Q

What should you consider when doing strength training with pt’s with ALS?

A
  1. Mindful of overuse fatigue if < 3/5
  2. Increased cramping, twitching, fatigue, DOMs
  3. > 3/5 will have moderate gains
  4. Avoid strenuous eccentric exercise
77
Q

What are three considerations for cardiovascular training in ALS pt’s?

A
  1. Recommended at mod-low intensities
  2. Improves heart and lung health and muscle efficiency
  3. Shorter bouts of exercise throughout the day may be most beneficial
78
Q

What are three recommendations for restorative or preventative interventions for ALS?

A
  1. Moderate to lot intensity exercise is tolerated well
  2. May improve strength, function, and early respiratory function in early phases
  3. Balance overuse fatigue with disuse atrophy
79
Q

What are common secondary impairments from muscle weakness in ALS?

A

Head falling forward, decrease ROM, contracture, subluxation, adhesive capsulitis, ambulation difficulties, deconditioning, impaired postural control

80
Q

What are examples of preventative interventions for ALS?

A

ROM to avoid contractures, pressure relief strategies, pulmonary care, balance and postural control

81
Q

What are examples of compensatory interventions for ALS?

A

Plan ahead for adaptive equipment, teach energy conservation strategies, caregiver education and training

82
Q

What compensatory strategies can be used for UE weakness?

A

Using a sling to support GH joint, hand and wrist splinting, adaptive equipment

83
Q

Would a pt with ALS benefit more from an AFO or KAFO?

A

AFO because it is more lightweight

84
Q

Would a pt with ALS benefit more from a rolling walker or a standard walker?

A

Rolling because they would not have to pick up the device

85
Q

What are some effects of exercise in pt’s with ALS?

A

Improved functional ability, cardiopulmonary function, vital capacity, slow down of motor neuron degeneration, improve strength in early stages

86
Q

Does exercise lead to improvements in QOL or survival with ALS?

A

No

87
Q

Practice Question: Which of the following is a possible concern a PT needs to be cautious of when prescribing exercise with a pt who has ALS?

a. muscle hypertrophy
b. muscle atrophy
c. overuse fatigue
d. excess oxygenation

A

c. overuse fatigue

88
Q

What two diseases effect the motor unit?

A

ALS and Post-Polio Syndrome

89
Q

What is Polio?

A

Virus invades cell bodies of LMN in ventral horn of the spinal cord that results in asymmetric flaccid paresis or paralysis

90
Q

What two conditions affect anterior horn cells of the spinal cord?

A

ALS and Post-Polio Syndrome

91
Q

What is Post-Polio Syndrome?

A

New neuromuscular symptoms occurring decades after recovery from acute paralytic episode

92
Q

When does Post-Polio develop?

A

About 25 years after initial recovery

93
Q

What % of people who had Polio will develop Post-Polio Syndrome?

A

25-50%

94
Q

What is the four main reasons for Post-Polio Syndrome?

A
  1. Denervated muscles were reinnervated by collateral sprouting
  2. Axons innervating more muscle fibers than originally intended
  3. Nervous system can no longer support giant motor units
  4. Prune back axonal sprouts
95
Q

What is the criteria for diagnosis of Post-Polio Syndrome?

A

Pt history or Polio, gradual onset of progressive and persistent new muscle weakness, pain, fatigue that lasts for at least one year

96
Q

What motor neuron signs are present in Post-Polio Syndrome?

A

LMN only

97
Q

What LMN signs are present with Post-Polio?

A

New muscle weakness, severe atrophy, hypotonia, fasiculations, pain, fatigue, hypoventilation, dysphagia, cold intolerane

98
Q

What medical interventions are indicated for Post-Polio Syndrome?

A

Pain management, anti-inflammatories, high doses of prednisone, immunoglobulin therapy, surgery to manage joint deformities, ventilation

99
Q

What are exercise recommendations and precautions for Post-Polio rehab?

A

Mod-low intensity regular exercise is recommended. Exercise should not cause lasting fatigue, increased pain. Should progress slowly

100
Q

What are other intervention goals for managing Post-Polio?

A

Improve energy efficiency, decrease joint pain, respiratory strengthening, adaptive strategies, weight management

101
Q

Practice Question: Post-Polio usually occurs in individuals with a priory history of poliomyelitis how many years ago?

a. 1-3
b. 5-10
c. 10-15
d. 20+

A

d. 20+

102
Q

Practice Question: Which of the following symptoms is not a common clinical presentation in patients with Post-Polio?

a. muscle atrophy
b. spasticity
c. fatigue
d. hyporeflexia

A

b. spasticity

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