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NMR II > Other Neuromuscular Diseases > Flashcards

Other Neuromuscular Diseases Flashcards

1
Q

What does ALS affect?

A
  • UMN
  • LMN
  • Cranial nerves (bulbar)
  • motor neurons in the spinal cord, brainstem & brain
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2
Q

What does ALS stand for?

A
  • Amyotrophy (muscle atrophy)
  • Lateral (Involving lateral & anterior corticospinal tracts)
  • Sclerosis (glial cell proliferation & hardening)
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3
Q

What is the main etiology of ALS?

A
  • Sporadic (90%)
  • Familial (10%)
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4
Q

ALS causes destruction of Upper Motor Neurons what is affected in cerebral cortex?

A
  • Pyramidal cells
  • Corticospinal tract
  • Corticobulbar tract
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5
Q

ALS causes destruction of Lower Motor Neurons what alpha motor neurons are affected?

A
  • anterior horn of spinal cord
  • Cranial nerve nuclei in brainstem
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6
Q

What are some other possible areas that can be impacted by neuronal loss when they have ALS?

A
  • Frontotemporal cortex
  • Thalamus
  • Basal ganglia/substantia nigra
  • Spinocerebellar tracts
  • Dorsal columns
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7
Q

What are some lower motor neuron symptoms in ALS?

A
  • Asymmetric (usually distal weakness)
  • Extensors weaker than flexors
  • Cervical extensor weakness (head drop)
  • Bulbar signs (CN IX-XII affected at nuclei or nerve)
  • Hyporeflexia
  • Hypotonicity
  • Atrophy
  • Muscle cramps
  • Fasciculations
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8
Q

What are some upper motor neuron symptoms in ALS?

A
  • Spasticity
  • Hyperreflexia/Clonus
  • Pathological reflexes (Babinski, Hoffman)
  • Muscle weakness (Extensors UE, Flexors LE)
  • Pseudobulbar palsy (corticobulbar tract)
  • as disease progresses, UMN signs may decrease
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9
Q

What is the presentation of bulbar symptoms in ALS?

A
  • Dysarthria
  • Dysphagia
  • Sialorrhea
  • Pseudobulbar affect (UMN)
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10
Q

What are the most common crania; nerve nuclei affected in ALS?

A
  • V
  • VII
  • IX
  • X
  • XII
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11
Q

What areas are preserved in clinical manifestation of ALS?

A
  • Eye movement
  • Bowel & Bladder
  • Sensory system
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12
Q

ALS has a variable presentation what are the early signs?

A
  • Insidious asymmetrical weakness of distal aspect of one limb
  • Cramping w/ volitional movement
  • Muscle fasciculations
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13
Q

How can ALS be diagnosed?

A

EMG

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14
Q

What is the first FDA approved drug for ALS?

A
  • Riluzole
  • May have neuroprotective effect but not cure
  • Slows progression
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15
Q

What is the prognosis of ALS?

A
  • Onset before 50 may have slower progression
  • Survival 2-5 yrs of sx onset
  • Bulbar onset more rapidly progressive
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16
Q

What is the average survival after symptoms onset of ALS?

A

3-5 years

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17
Q

What does the Amyotrophic Lateral Sclerosis Functional Rating Scale Revised examine? What does lower score indicate?

A
  • Evaluates 12 areas of function
  • Lower score = worse function
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18
Q

What does ALS Assessment Questionnaire - 40 examine and what do higher scores indicate?

A
  • QOL assessment
  • Higher score = more impairment
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19
Q

What is included in palliative approach to care?

A
  • SLP consult
  • Respiratory issues
  • Pain
  • Mental health
  • Cognitive implications
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20
Q

T/F: Patients with ALS may have qsialorrhea & dysphagia

A

True
- Require nutrition management, eventually may need PEG tube

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21
Q

Patients with ALS may have dysarthria. What is used to combat these issues?

A
  • Speech language pathologist is involved
  • Use of speech strategies & environmental modifications
  • Use of devices
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22
Q

What patients with ALS vital capacity drops below 50% what is recommended?

A
  • Non-invasive positive pressure ventilation is recommended
  • CPAP, BiPAP, Supplemental O2 not recommended unless coexisting pulmonary disease
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23
Q

When non-invasive ventilation is no longer affected what is considered?

A
  • Invasive ventilation
  • Hospice care
24
Q

How can muscle cramps and spasticity be managed in patient with ALS?

A
  • Good hygiene
  • Stretching problems
  • Medical management (anticonvulsants for cramps, benzodiazepines for spasticity, NSAIDs/opios for pain, Ativan for fasciculations
25
Q

How is anxiety & depression managed in patients with ALS?

A
  • Counseling
  • Pharmacologic intervention (SSRI, Tricyclic antidepressants, Benzodiazepines)
26
Q

T/F: Prolonged survival of patients with ALS is seen in those with positive psychological well-being

27
Q

What dementia can be seen in patients with ALS?

A
  • ALS Frontotemporal Dementia (ALS-FTD)
28
Q

What impairments are seen in patients with ALS-FTD?

A
  • Cognitive decline
  • Executive function
  • Difficulty with planning, organization & concept abstraction
  • Personality & behavior changes
29
Q

Patients with ALS that do not have ALS-FTD have also demonstrated what impairments?

A
  • Difficulties with verbal fluency
  • Language comprehension
  • Memory
  • Abstract reasoning
  • Generalized impairments in intellectual function
30
Q

What can be used to help determine those with ALS-FTD?

A
  • ALS Cognitive Behavior Screen (ALS-CBS)
  • Assess cognition & behavior changes
31
Q

What are the goals of early rehab in patients with ALS?

A
  • Preventative/restoration
  • Introduce some compensatory techniques
32
Q

What are some goals of middle rehab in patients with ALS?

A
  • Compensatory/preventative
  • Less focus on restorative
33
Q

What are the goals of late rehab in patients with ALS?

A
  • Compensatory/preventative
  • Limited focus on restorative
34
Q

In early phases of ALS, reinnervation can compensate for loss of strength when can reinnervaiton no longer compensate?

A

Once 50% of the motor units have been lost

35
Q

T/F: Exercise can prolong survival in patients with ALS

36
Q

What are the benefits of exercise in ALS?

A
  • Stress reduction
  • Maintaining strength as long as possible
  • Stay flexible as body becomes more sedentary
  • Improving sleep
  • Max respiratory & cardiac function
37
Q

What should you be mindful of when strengthening patients with ALS?

A

Overuse fatigue in muscles graded <3/5

38
Q

T/F: Strenuous eccentric exercise in appropriate for patients with ALS

A

False -avoid it

39
Q

In patients with ALS, when should moderate strengthening exercises should be performed, and strength gains can be expected?

A

Muscles >3/5

40
Q

What type of cardiovascular training should be performed in patients with ALS? What does it improve?

A
  • Moderate- low intensities
  • Shorter bouts of exercises throughout the day may be beneficial
  • Improves heart & lung health and muscle efficiency
41
Q

In ALS motor neuron degeneration results in muscle weakness what are the secondary impairments?

A
  • Head falls forward
  • Decreased ROM, tendon shortening, contractures
  • Shoulder sublux
  • Adhesive capsulitis
  • Ambulation difficulties
  • Deconditioning
  • Impaired postural control
42
Q

Patients with ALS have greater loss of mm strength in distal or proximal first?

A

Distal first

43
Q

What are some preventative PT interventions for patients with ALS?

A
  • ROM to avoid contractures
  • Pressure relief strategies as person becomes more dependent to maintain healthy skin integrity
  • Pulmonary care
  • Balance/postural control
44
Q

What are some compensatory PT interventions for patients with ALS?

A
  • Plan ahead for adaptive equipment (W/C, adaptation in home, ADL)
  • Teach energy conservation strategies
  • Caregiver education & training
  • Equipment to support weak cervical muscles
45
Q

What compensatory PT interventions can be used to address UE weakness in patients with ALS?

A
  • Consider using sling to support glenohumeral joint if the shoulder is painful from sublux
  • Hand & wrist splinting
  • Adaptive equipment
46
Q

Should an AFO or KAFO be used for patients with ALS? Should rolling walker or standard walker be used?

A
  • AFO
  • Rolling walker
47
Q

What compensatory PT interventions can be used to address gait in patients with ALS?

A
  • Early introduction of orthotics & AD to assist with energy conservation and in reducing falls
48
Q

What compensatory PT intervention can be used to address mobility in patients with ALS?

A
  • Raised surface heights for STS
  • Seat lifts to improve STS abilities
  • Pivot discs for transfers
  • Stair lifts
  • Mechanical lifts. hospital beds as needed
49
Q

What is polio?

A

Virus invades cell bodies of lower motor neurons in ventral horn of spinal cord resulting in asymmetric flaccid paresis or paralysis

50
Q

What is post-polio syndrome?

A

New neuromuscular symptoms occurring decades (average 25 yrs) after recovery from acute paralytic episode

51
Q

What is the etiology of post-polio syndrome?

A
  • Denervated muscles were reinnervated by collateral sprouting from surviving nerves
  • Axons innervating more muscle fibers than originally intended
  • Nervous system can no longer support giant motor units
  • Prone back axonal sprouts
52
Q

What is the criteria for diagnosis of post-polio syndrome?

A
  • History of confirmed prior poliomyelitis –> recovery –> gradual onset of new sx
  • sx persist a least a year
  • exclusion of any other condition that causes sx
53
Q

What are the symptoms of post-polio syndrome?

A
  • LMN signs only
  • New/exacerbated muscle weakness
  • Severe muscle atrophy
  • Hypotonia
  • Fasciculations
  • Pain
  • Fatigue
  • Hypoventilation
  • Dysphagia
  • Cold intolerance
54
Q

What medical interventions can be performed in patients with post-polio syndrome?

A
  • Pain management
  • Anti-inflammatories
  • Maybe surgery
  • Ventilatory support if needed
55
Q

What is done as rehab management for patients with post-polio syndrome?

A
  • Those who exercise regularly may experience fewer sx & higher level of function
  • Moderate-low intensity regular exercise (low reps while ensuring appropriate demand)
56
Q

What are some exercise precautions for patients with post - polio syndrome?

A
  • Exercise should not cause lasting fatigue
  • Exercise should not causes increased pain/soreness
  • Progress exercises slowly with monitoring
57
Q

What is the goal of rehab management in patients with post-polio syndrome?

A
  • Improve energy efficiency by reducing overwork
  • Decrease joint pain
  • Include respiratory strengthening
  • Recommend adaptive strategies when appropriate
  • Weight management

NMR II (14 decks)

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