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Neuro 2 > ALS > Flashcards

ALS Flashcards

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&raquo_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

A

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

21
Q

What type of ALS is more common in females?

A

bulbar onset classical ALS

22
Q

What type of ALS is more common in males?

A

Progressive spinal muscular atrophy (SMA IV)

23
Q

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

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?

A
  • symmetrical “Man in barrel syndrome”
  • Characterized by severe muscle wasting/atrophy of both arms
  • Progressive Spinal Muscular Atrophy (SMA IV)
25
Q

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

A
  • Severe flaccid paralysis of legs
  • can be unilateral and bilateral presentation
  • Progressive Spinal Muscular Atrophy (SMA IV)
26
Q

What is the progression of Progressive Spinal Muscular Atrophy (SMA IV)?

A
  • highly variable
  • can be slow or very rapid
27
Q

What are common cognitive deficits with ALS? What does this causes problems with?

A

Common deficits - executive function, either language or personality

Causes problems with - judgement, impulsivity, decreased ability to handle routine tasks

28
Q

age of onset of familial ALS

A

late 40s or early 50s

29
Q

characteristics of stage 1 ALS

A
  • early disease with mild focal weakness
  • asymmetrical distribution
  • symptoms of hand cramping and fasciculations
30
Q

characteristics of stage 2 ALS

A
  • moderate weakness in groups of muscles
  • some wasting (atrophy) of muscles
  • ModI with AD
31
Q

characteristics of stage 3 ALS

A
  • severe weakness of specific muscles
  • increasing fatigue
  • mild to moderate functional limitations
  • ambulatory
32
Q

characteristics of stage 4 ALS

A
  • severe weakness and wasting of LEs and mild weakness of UEs
  • moderate AD and wheelchair user
33
Q

characteristics of stage 5 ALS

A
  • 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
Q

characteristics of stage 6 ALS

A
  • bedridden
  • dependent ADLs
  • FMS: progressive respiratory distress
35
Q

What is the ALS Functional Rating Scale? What is the total possible score?

A
  • 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
Q

prognosis of ALS

A
  • mortality rate 2-5 years
  • ultimate progress to respiratory failure
37
Q

negative prognostic indicators

A
  • bulbar-onset
  • early respiratory signs
38
Q

positive prognostic indicators

A
  • patients who participate in multidisciplinary clinics live longer and have an improved QoL
39
Q

What airway clearance technique has proven long term benefit?

A

inspiratory muscle training

40
Q

What airway clearance recruitment training has proven immediate benefit?

A

lung volume recruitment training

41
Q

ALS exercise program

A

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
Q

Exercise precautions with ALS

A
  • 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
Q

PT considerations for stage 1 ALS

A

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
Q

PT considerations for stage 2 ALS

A

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
Q

PT considerations for stage 3 ALS

A

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
Q

PT considerations for stage 4 ALS

A

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
Q

PT considerations for stage 5 ALS

A

Stage 5 - progressive weakness of mobility and endurance
- inability to transfer
- pain contracture and cramping
- maintenance program: stretching, splinting, STM, orthotics

48
Q

PT considerations for stage 6 ALS

A

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
Q

What is the most common type of ALS in older females?

A

bulbar onset ALS

Neuro 2 (19 decks)

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