Neuro 2 > ALS > Flashcards

ALS Flashcards

(49 cards)

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1
Q

risk factors for ALS

A
  • male > female
  • white, non-hispanics minorities
  • geographical clusters
  • family history
  • age (55-75)
  • prior trauma or TBI
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2
Q

____________ levels are higher in patients with limb-onset ALS

A

creatine phosphokinase

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3
Q

What is required for clinical diagnosis of ALS?

A

pattern of observed and reported symptoms of both UMN and LMN as well as persistent decline in physical function that cannot be attributed to other disorders

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4
Q

El Escorial Criteria for Diagnosis of ALS

A

Exclusion of all other Dx + Progressive Functional Decline AND
- Progressive UMN and LMN deficits in >/= 1 region of the human body
OR
- LMN deficits in >/= 1 limb/region as defined by clinical examination and/or by EMG in 2 clinical regions

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5
Q

The Gold Coast Criteria for Diagnosis of ALS

A
  • Exclude other disease AND
  • Progressive motor impairment documented by hx or repeated clinical assessment, proceeded by normal motor function AND
  • Presence of UMN and LMN signs in >/= region or LMN dysfunction in >/= 2 regions
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6
Q

What is the most common impairment of ALS?

A

focal, asymmetrical muscle weakness beginning in LE or UE, or bulbar weakness
- LMN weakness»_space; UMN weakness

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7
Q

What muscles are usually spared until terminal stages?

A
  • eye
  • bowel and bladder
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8
Q

common UMN impairments

A
  • spasticity
  • hyperreflexia
  • clonus
  • pathological reflexes
  • weakness
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9
Q

LMN impairments

A
  • hyporeflexia
  • hypotonicity
  • atrophy
  • fasciculations
  • weakness
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10
Q

What is bulbar weakness?

A

lower cranial nerves that control talking, swallowing, chewing (mouth, throat and neck)

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11
Q

Bulbar impairments

A
  • spastic or flaccid bulbar palsy
  • dysarthria
  • dysphagia
  • sialorrhea (excessive salivation)
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12
Q

What are early S&S of respiratory impairments?

A
  • fatigue
  • dyspnea on exertion
  • difficulty sleeping in supine
  • frequent awakening at night/excessive daytime sleeping
  • recurring sighing due to lack of O2
  • morning headaches
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13
Q

Vital capacity of ________ = significant risk of impending respiratory failure or death

A

25-35%

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14
Q

What is typically spared for most of the disease course?

A
  • sensory pathways
  • ocular motor function
  • sphincter (bowel/bladder) control
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15
Q

What are early signs of limb onset classical ALS? bulbar classical ALS

A
  • progressive limb weakness
  • reduced dexterity
  • wasting of hands
  • muscle fasciculations

Bulbar
- wasting of tongue and muscles for speech and swallowing
- and all above signs

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16
Q

What causes the first symptoms of limb-onset classical ALS?

A

damage to motor neurons in motor cortex, specifically corticospinal tracts

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17
Q

What causes the first symptoms of bulbar-onset classical ALS?

A

damage to motor neurons in brainstem, specifically corticobulbar tracts (face, head, necks muscles)

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18
Q

What are some examples of first symptoms seen with bulbar-onset?

A
  • CN IX and XII causing dysarthria and dysphagia
  • tongue atrophy, tongue fasciculations
  • vocal cord spasms
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19
Q

S&S of primary lateral sclerosis

A

UMN ONLY
- spasticity
- UMN pattern weakness
- pseudobulbar findings

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20
Q

Prognosis of primary lateral sclerosis

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A

relatively good with a life expectancy comparable to normal
- slower degeneration

21
Q

What type of ALS is more common in females?

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A

bulbar onset classical ALS

22
Q

What type of ALS is more common in males?

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A

Progressive spinal muscular atrophy (SMA IV)

23
Q

S&S of Progressive Spinal Muscular Atrophy (SMA IV)

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A

LMN ONLY
- flaccid weakness
- atrophy
- fasciculation
- hyporeflexia or areflexia

24
Q

What is flail arm syndrome? Which type of ALS is it associated with?

Study These Flashcards
A
  • symmetrical “Man in barrel syndrome”
  • Characterized by severe muscle wasting/atrophy of both arms
  • Progressive Spinal Muscular Atrophy (SMA IV)
25
What is flail leg syndrome? Which type of ALS is it associated with?
- Severe flaccid paralysis of legs - can be unilateral and bilateral presentation - Progressive Spinal Muscular Atrophy (SMA IV)
26
What is the progression of Progressive Spinal Muscular Atrophy (SMA IV)?
- highly variable - can be slow or very rapid
27
What are common cognitive deficits with ALS? What does this causes problems with?
Common deficits - executive function, either language or personality Causes problems with - judgement, impulsivity, decreased ability to handle routine tasks
28
age of onset of familial ALS
late 40s or early 50s
29
characteristics of stage 1 ALS
- early disease with mild focal weakness - asymmetrical distribution - symptoms of hand cramping and fasciculations
30
characteristics of stage 2 ALS
- moderate weakness in groups of muscles - some wasting (atrophy) of muscles - ModI with AD
31
characteristics of stage 3 ALS
- severe weakness of specific muscles - increasing fatigue - mild to moderate functional limitations - ambulatory
32
characteristics of stage 4 ALS
- severe weakness and wasting of LEs and mild weakness of UEs - moderate AD and wheelchair user
33
characteristics of stage 5 ALS
- progressive weakness with deterioration of mobility and endurance - increased fatigue - moderate to severe weakness of whole limbs and trunk - spasticity - hyperreflexia - loss of head control - max assist
34
characteristics of stage 6 ALS
- bedridden - dependent ADLs - FMS: progressive respiratory distress
35
What is the ALS Functional Rating Scale? What is the total possible score?
- measures ADL and global function across 12 functional categories - used to assess response to treatment or disease progression - total score of 48 with lower scores = worse performance
36
prognosis of ALS
- mortality rate 2-5 years - ultimate progress to respiratory failure
37
negative prognostic indicators
- bulbar-onset - early respiratory signs
38
positive prognostic indicators
- patients who participate in multidisciplinary clinics live longer and have an improved QoL
39
What airway clearance technique has proven long term benefit?
inspiratory muscle training
40
What airway clearance recruitment training has proven immediate benefit?
lung volume recruitment training
41
ALS exercise program
Best done during the early stages - mild resistive exercise if muscles are good to normal ranges (3-5) - mild aerobic activities at sub max levels (60-75% HRmax, 10-20 min, 3x wk
42
Exercise precautions with ALS
- monitor fatigue closely - avoid overwork injury - avoid exercise if less than 1/3 of motor units are functioning - limited positions with decreased pulmonary function
43
PT considerations for stage 1 ALS
Stage 1 - independent of mobility and ADLs - continue normal activities - stretch affected joints - AROM, resistive exercises to unaffected muscles - aerobic activities - moderate intensity to tolerance
44
PT considerations for stage 2 ALS
Stage 2 - moderate weakness in groups of muscles - muscle weakness in extremities can result in difficulty with ADLs - assess for use of AD - continue stage 1 activities with more conservation techniques - increased need for caregiver assistance - Exercise 2-3x wk w/ rest in between - airway clearance techniques
45
PT considerations for stage 3 ALS
Stage 3 - continued ambulation but severe weakness, SOB with exertion and/or sleep - paralysis occurs in some muscles - Spasticity increases as more LMN are damaged - keep pt physically independent - energy conservation techniques crucial - use of splints, orthotics, AD
46
PT considerations for stage 4 ALS
Stage 4 - severe widespread (UE/LE) weakness, augmented communication due to weak speaking muscles - wheelchair level - continue P/AROM to prevent contractures - strengthening therex to major muscle groups - increased focus on preventing skin complications - ordering necessary equipment
47
PT considerations for stage 5 ALS
Stage 5 - progressive weakness of mobility and endurance - inability to transfer - pain contracture and cramping - maintenance program: stretching, splinting, STM, orthotics
48
PT considerations for stage 6 ALS
Stage 6 - bedridden, respiratory muscle weakness requiring mechanical vent - max assist to dependent for all mobility - skin care and prevention - pain management - postural drainage - coughing techniques - airway clearance
49
What is the most common type of ALS in older females?
bulbar onset ALS
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