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ALS Flashcards

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1
Q

What is ALS?

A

neurodegenerative condition hat affects motor function, leading to progressive muscle weakness and wasting

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2
Q

Does ALS has UMN or LMN signs?

A

Both
*LMN as the disease progresses

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3
Q

Anatomy affected in ALS

A
  • frontal motor neurons w/UMN degeneration
  • axonal degeneration and gliosis in the pyramidal tracts (corticospinal/corticobulbar)
  • degeneration of frontal motor neurons leads to LMN degeneration
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4
Q

Common signs of LMN involvement in ALS? (3)

A
  • weakness
  • atrophy
  • fasciculations
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5
Q

What are the 3 subgroups of ALS?

A
  1. Limb onset
  2. Bulbar onset
  3. Respiratory onset
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6
Q

Key features of limb onset ALS (2)

A
  • 75-80% of cases
  • weakness and clumsiness
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7
Q

Key features of bulbar onset ALS (2)

A
  • 20-25% of cases
  • dysarthria and drooling
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8
Q

Key features of respiratory onset ALS (3)

A
  • <3% of cases
  • dyspnea on exertion
  • orthopena
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9
Q

Symptoms of limb onset ALS (9)

A
  • PAINLESS mm weakness
  • clumsiness
  • impaired fine motor skills
  • limb stiffness
  • gait changes
  • foot drop/wrist drop
  • weight loss
  • FASCICULATIONS
  • mm cramps
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10
Q

Symptoms of bulbar onset ALS (6)

A
  • dysarthria
  • voice change
  • pseudobulbar affect
  • dysphagia > weight loss
  • drooling
  • choking during swallowing
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11
Q

Symptoms of respiratory onset ALS (4)

A
  • dyspnea on exertion
  • dyspnea at rest
  • orthopnea
  • dyssomnia/daytime sleepiness
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12
Q

Additional symptoms of ALS (3)

A
  • cog changes (development of frontotemporal dementia)
  • sensation and ANS function is SPARED
  • bowel and bladder continences is PRESERVED
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13
Q

Epidemiology of ALS (6)

A
  • M > F
  • more common in European heritage
  • sporadic ALS (90%)
  • environmental exposure (military)
  • tobacco use
  • familial ALS (5-10%)
    *SOD1 variant = 50% chance ea offspring will inherit ALS gene
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14
Q

ALS Prognosis (4)

A
  • 50% of patient die within 30 months of sx onset
  • 20% survive for 5 years
  • 10% survive for 10 years
  • 5% live 20 years or longer
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15
Q

Poor prognostic factors of ALS (4)

A
  • lower vital capacity at diagnosis
  • older age at onset
  • bulbar subtype
  • increased psychological distress
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16
Q

Positive prognostic factors of ALS (1)

A
  • longer delay from symptom onset to diagnosis
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17
Q

What are motor neuron diseases that may present like ALS? (3)

A
  • Primary lateral sclerosis
  • Progressive muscular atrophy
  • Kennedy’s disease
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18
Q

What at non-motor diseases that may present like ALS? (4)

A
  • lumbar radiculopathy (myelopathy)
  • MS
  • brain tumor
  • hereditary spastic paraplegia
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19
Q

What is Primary Lateral Sclerosis (PLS)?

A

Rare, non-hereditary, idiopathic, SLOW, progressive, UMN degenerative disorder

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20
Q

Symptoms of Primary Lateral Sclerosis (6)

A
  • spasticity
  • hyper-reflexia
  • weakness
  • dysphagia
  • dysarthria
  • pseudobulbar effect
    *slowly spread from either top down or bottom up
    *similar sx to ALS, but UMN only
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21
Q

Epidemiology of Primary Lateral Sclerosis (3)

A
  • 2-5% of adults
  • age of onset ~50 years
  • males > females
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22
Q

Prognosis of Primary Lateral Sclerosis (2)

A
  • overall positive d/t slow progression and late onset
  • allows pt to maintain LMN function
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23
Q

Treatment of Primary Lateral Sclerosis (3)

A
  • no medical or surgical management aside from supportive care
  • PT interventions similar to those for ALS
  • focus on spasticity/contracture management and strength
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24
Q

What is Progressive Muscular Atrophy (PMA)?

A

Rare motor neuron disease affecting only the lower motor neurons (LMNs)

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25
What are the demographics and etiology of Progressive Muscular Atrophy (PMA)? (3)
- > 50 years old - men > women - environmental and/or genetic factors
26
Symptoms of Progressive Muscular Atrophy (PMA)? (6)
- muscle atrophy - axial and appendicular skeleton - fatigue - fasciculations - clumsiness - breathing difficulties - weight loss
27
Prognosis of Progressive Muscular Atrophy (PMA)? (2)
- slowly progressive - from time of diagnosis to death is > 5 years
28
Treatment of Progressive Muscular Atrophy (PMA)? (3)
- no medical or surgical management aside from supportive care - PT interventions similar to those for ALS - focus on adaptive equipment, activity modification/fatigue management
29
What is Kennedy's Disease?
Rare, x-linked recessive, genetic, progressive adult-onset motor neuron disease *AKA: Spinobulbar Muscular Atrophy (SBMA)
30
Epidemiology of Kennedy's Disease? (2)
- 3.3/100,000 males (rarely affects females) - 30-60 years old
31
Anatomy/Physiology of Kennedy's Disease
Due to a misfolding of androgen receptor proteins causing an aggregation in the motor neurons and degeneration of anterior horn cells - anterior horn cells exit to spinal cord to produce muscle motion
32
Symptoms of Kennedy's Disease (10)
*both spinal and bulbar mm affected - weakness of facial, bulbar, and extremity mm - absent DTRs - mm cramps - fasciculations - jaw drop - dysarthria - dysphagia - gynecomastia - fertility challenges - diabetes
33
Prognosis of Kennedy's Disease (3)
- slowly progress - lifespan is normal - ambulatory until later stage disease - cause of death usually respiratory/pneumonia
34
Treatment of Kennedy's Disease (3)
- Medical: androgen reduction therapies, gonadotropin releasing hormone therapy - Allied Health: PT, OT, SLP - PT focusing on mm strength, posture, and reducing fall risk
35
ALS Diagnostic Criteria
*must have all 3 1. Progressive motor impairment via medical history or repeated clinical assessment w/previous normal motor function 2. Presence of UMN and LMN dysfunction in at least 1 body region (same body region) or LMN in at least 2 body regions 3. Investigations excluding other disease process
36
What testing can aide in the diagnosis of ALS? (2)
- EMG and nerve conduction studies (NCV) - MRI (T2 hyper intensity of corticospinal and/or corticobulbar tracts)
37
ALS EMG findings (3)
- Fibrillation potentials indicating acute/recent denervation - visible and audible prolonged and disorganized motor units reflecting chronic denervation - fasciculations
38
ALS NCV findings (3)
- reduced amplitudes - NCV often preserved until amplitudes are severely reduced - sensory nerves are normal
39
How is ALS classified? (4)
1. Stage of disease 2. Phenotype 3. Diagnosis 4. El Escorial Category
40
ALS Staging (King Staging) - Stage 1
Symptom onset (clinical involvement of first anatomic region)
41
ALS Staging (King Staging) - Stage 2A
Diagnosis
42
ALS Staging (King Staging) - Stage 2B
Involvement of second anatomic region
43
ALS Staging (King Staging) - Stage 3
Involvement of third anatomic region
44
ALS Staging (King Staging) - Stage 4A
Need for gastrostomy
45
ALS Staging (King Staging) - Stage 4B
Need for noninvasive ventilation
46
ALS Phenotyping
1. Limb 2. Bulbar 3. Respiratory
47
ALS El Escorial Criteria - Possible ALS
Presence of UMN and LMN signs in one region OR UMN signs in 2-3 regions, such as monomelic ALS, progressive bulbar palsy, and primary lateral sclerosis
48
ALS El Escorial Criteria - Probable ALS, lab results supported
Presence of UMN and LMN signs in one region w/evidence by EMG of LMN involvement in another region
49
ALS El Escorial Criteria - Probable ALS
Presence of UMN and LMN signs in at least 2 regions w/UMN signs rostral to LMN signs
50
ALS El Escorial Criteria - Definite ALS
Presence of UMN and LMN signs in 3 anatomical Regions
51
ALS El Escorial Criteria Categories
1. Definite ALS 2. Probable ALS 3. Probable ALS, laboratory results supported 4. Possible ALS
52
Medical Management of ALS
Riluzole (RILUTEK) - only approved drug for ALS treatment - mod survival effects (delays median time of death) - neuroprotective benefits for motor neurons - enteric - pill form or liquid
53
Commonly used outcome measures (5)
5xSTS 10MWT TUG BBS vs. FGA Modified Fatigue Impact Scale
54
What is the ALS functional rating scale (ALSFRS-R)?
Test that measures ADLs and global function in ALS patients - 12 question scale (0-4) - higher score = better/more functional - assesses gross motor and fine motor function
55
MDC for ALS functional rating scale (ALSFRS-R)?
6 pts
56
Evidence Based PT for ALS
1. For groups performing 2x/d vs. 3x/wk mod intensity exercise > both show sig improvement in ALSFRS- R; no sig diff in QoL, fatigue, or mm strength *evidence to small to determine benefit vs. harm
57
Evidence Based PT for ALS - can therEx slow down progressive functional decline in patients w/ALS?
- lack of clinical trials and data outside of ALSFRS scores - low quality studies - non uniform exercise interventions Results: min adverse events (good safety) - ALSFRS scores at 6 months less than control groups (reduced rate of decline) - exercise not shown to improve strength of mm already weakened by ALS - encourage multi-modal management program (strength and aerobic)
58
ALS PT Treatment - Early Stage (4)
AVOID OVERWORKING MM - stretching and ROM to prevent contractures - Moderate resistance training (in mm > 3/5 MMT) - Aerobic exercise in mild-mod level
59
Signs of "overworking mm" (4)
- fatigue - cramping - twitching - soreness
60
ALS patient presentation in early stage
Independent w/mobility, function, ADLs
61
ALS PT Treatment - Middle Stage (5)
- stretching, massage, and ROM for pain management - maintain mobility efficiently (cane, walker, AFOs, neck brace) - fall prevention strategies and balance exercises - recommendation of adaptive equipment (grab bars, shower/toilet modifications) - initiate breathing exercises to optimize respiratory function
62
ALS patient presentation in middle stage
Increased number and severity of impairments - progressive decline in function > compensatory measures
63
ALS PT Treatment - Late Stage (4)
- optimize comfort w/seating systems/air mattress - consider wheelchairs/power mobility *Goals: prevent pressure ulcerations, support pt in upright position for optimizing breathing and swallowing - PROM or massage for pain management - caregiver training (lift system education, wheelchair mobility training)
64
ALS patient presentation in late stage (4)
Totally dependent for mobility and function - Dysarthria - Dysphagia - Respiratory disease
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