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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
Q

Intervention Goals

A

-maintain fullest physical, psychological and social function and independence for as long as possible

26
Q

Unrealistic Goals

A
  • Increase strength
  • prevent strength loss over long-term
  • alter disease course
27
Q

Intervention Approaches

A
  • Restorative
  • Preventative
  • Compensatory
28
Q

Stages of ALS

A

-I through VI

29
Q

Early Disease Challenges

A
  • emotional impact of diagnosis
  • uncertain future
  • inevitable loss
30
Q

How Denial can be beneficial

A
  • might help accept horrible diagnosis easier

- digest it in smaller chunks

31
Q

Middle Disease Challenges

A
  • multiple losses experienced
  • great need to make decisions
  • may be reluctant to use compensatory strategies
32
Q

Late Disease Challenges

A
  • confronted with terminal aspects of disease
  • worries about family/loved ones
  • decisions regarding life-saving measures
33
Q

Psychological Support

A
  • Education
  • stage appropriate hope
  • reassure
  • caution with web searches
  • interdisciplinary approach
  • give opportunities for decision making
  • emphasize abilities
34
Q

Remediation

A
  • strengthening
  • stretching
  • aerobic conditioning
  • leisure/recreation
35
Q

Prevention

A
  • pressure
  • respiratory
  • spasticity
  • DVT
  • contractures
  • ROM
  • bracing
  • positioning
36
Q

Compensation

A
  • AD
  • orthotics
  • respiratory aids/vents
  • home modification
  • caregiver training
37
Q

Spasticity Management

A
  • meds: limited use
  • stretching
  • positioning
  • exercise
38
Q

Strengthening Guidelines

A
  • 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
Q

Signs of Respiratory Mm Weakness

A
  • dyspnea
  • orthopnea
  • sleep disturbance
  • daytime hypersomnolence
  • morning headaches
  • abnormal breathing patterns
40
Q

Leading cause of death

A

-respiratory failure

41
Q

Managing Secretions

A
  • suction catheters
  • assisted or mechanical coughing techniques
  • pulmonary hygiene methods
  • tracheostomy
42
Q

Trach Tubes

A
  • QoL better for pts

- QoL worse for caregivers (lost jobs, lack of sleep to care for pt etc)

43
Q

Maintaining Oxygen

A

-non-invasive positive pressure

44
Q

Tracheostomy/Mechanical Ventilation

A

-

45
Q

Palliative Care

A

-

Neuromuscular Conditions II (15 decks)

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