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Neuromuscular Conditions II > ALS > Flashcards

ALS Flashcards

1
Q

most common form of _____ disease

A

-motor neuron

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2
Q

more common in ____

A
  • males (below age 50)

- after age 50, males=females

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3
Q

Forms of ALS

A
  • Classical Sporadic (most common)
  • Pacific (guam)
  • Familial (genetic)
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4
Q

Cause of ALS

A
  • unknown
  • environmental (diet?)
  • trauma
  • physical/emotional stress
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5
Q

Average Onset Age

A

-60 years

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6
Q

Onset

A

-typically starts in one limb

  • Upper Limb
  • Lower Limb
  • Bulbar (CN S/Sx)
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7
Q

Clinical Course

A

-progressive deterioration

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8
Q

Bulbar signs

A
  • poor prognosis

- CN symptoms/signs

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9
Q

Life expectancy

A

-2-5 years from diagnosis

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10
Q

Riluzole

A

-drug extends life by about 3 months

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11
Q

Pathology

A
  • loss of selective motor neurons

- UMN and LMN signs

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12
Q

CNS Areas Affected

A
  • Cortex, brainstem, spinal cord
  • frontotemporal cortex (mental changes)
  • posterior columns (sensory)
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13
Q

Mixed UMN & LMN signs

A
  • UMN: spasticity

- LMN: flaccidity

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14
Q

collateral sprouting can cause

A
  • reinnervation

- not as effective as other diseases

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15
Q

UMN S/Sx

A
  • spasticity
  • hyperreflexia
  • loss of dexterity & speed
  • pathological reflex
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16
Q

LMN S/Sx

A
  • weakness/atrophy
  • hyporeflexia
  • fasciculations
  • muscle cramps
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17
Q

Bulbar related S/Sx

A
  • speech/swallowing
  • enhanced gag reflex
  • suck/snout reflexes
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18
Q

Cognitive Involvement:

Frontotemporal

A
  • verbal/nonverbal fluency
  • abstract reasoning
  • memory for verbal and nonverbal material
  • working memory
  • mental flexibility
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19
Q

Activity Limitations

A
  • moility
  • ADL/IADL
  • Speech production
  • Swallowing
  • Respiration

-Bowel/bladder spared

20
Q

Better QoL Associated with

A

-psychological and existential scales, religious faith and support

21
Q

QoL not associated with

A

-physical funciton/strength

22
Q

ALS

A

-amyotrophic lateral sclerosis

23
Q

PT Exam

A
  • cognition
  • pain
  • joint integrity/ROM
  • muscle performance
  • motor function
  • tone/reflexes
  • CN integrity
  • balance, gait, function
  • respiratory function
  • integumentary integrity
  • environmental barriers
  • endurance/fatigue
24
Q

ALS Functional Rating Scale

A
  • self-report

- assess various aspects of function

25
Intervention Goals
-maintain fullest physical, psychological and social function and independence for as long as possible
26
Unrealistic Goals
- Increase strength - prevent strength loss over long-term - alter disease course
27
Intervention Approaches
- Restorative - Preventative - Compensatory
28
Stages of ALS
-I through VI
29
Early Disease Challenges
- emotional impact of diagnosis - uncertain future - inevitable loss
30
How Denial can be beneficial
- might help accept horrible diagnosis easier | - digest it in smaller chunks
31
Middle Disease Challenges
- multiple losses experienced - great need to make decisions - may be reluctant to use compensatory strategies
32
Late Disease Challenges
- confronted with terminal aspects of disease - worries about family/loved ones - decisions regarding life-saving measures
33
Psychological Support
- Education - stage appropriate hope - reassure - caution with web searches - interdisciplinary approach - give opportunities for decision making - emphasize abilities
34
Remediation
- strengthening - stretching - aerobic conditioning - leisure/recreation
35
Prevention
- pressure - respiratory - spasticity - DVT - contractures - ROM - bracing - positioning
36
Compensation
- AD - orthotics - respiratory aids/vents - home modification - caregiver training
37
Spasticity Management
- meds: limited use - stretching - positioning - exercise
38
Strengthening Guidelines
- early stages only - don't strengthen muscles with grade <3/5 - aware of overuse signs - can strengthen unaffected Mm - short bouts (10 min, 2-3x/day)
39
Signs of Respiratory Mm Weakness
- dyspnea - orthopnea - sleep disturbance - daytime hypersomnolence - morning headaches - abnormal breathing patterns
40
Leading cause of death
-respiratory failure
41
Managing Secretions
- suction catheters - assisted or mechanical coughing techniques - pulmonary hygiene methods - tracheostomy
42
Trach Tubes
- QoL better for pts | - QoL worse for caregivers (lost jobs, lack of sleep to care for pt etc)
43
Maintaining Oxygen
-non-invasive positive pressure
44
Tracheostomy/Mechanical Ventilation
-
45
Palliative Care
-

Neuromuscular Conditions II (15 decks)

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