Chapter 2: Amyotrophic Lateral Sclerosis (ALS) - Lou Gehrig's Disease

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dmshaw9
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298338
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Chapter 2: Amyotrophic Lateral Sclerosis (ALS) - Lou Gehrig's Disease
Updated:
2015-03-13 14:57:37
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Amyotrophic Lateral Sclerosis
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NPTE,Chapter2
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NPTE: Chapter 2
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  1. Definition
    • A degenerative disease affecting UMN’s and LMN’s 
    • Degeneration of anterior horn cells and descending corticobulbar and corticospinal tracts 
  2. Characteristics
    • Progressive disease
    • Often leads to death (2-5 years)
    • Highly variable symptoms – Bulbar Onset and SC Onset
  3. Bulbar Onset 
    Progressive bulbar palsy
  4. SC Onset 
    Progressive muscular atrophy
  5. Signs and Symptoms
    • Muscular weakness: spreads over time (limbs → whole body)
    • Atrophy, cramping, muscle fasciculations or twitching (LMN signs)
    • Spasticity, hyperreflexia (UMN sign)
    • Dysarthria, dysphagia, dysphonia secondary to psuodbulbar palsy and progressive bulbar palsy 
    • Usually absence of sensory changes 
    • Autonomic dysfunction (~1/3 of pts)
    • Pain due to spasticity, cramping, postural stress syndrome, joint hypomobility, instability
    • Respiratory impairments = weakness > paralysis, nocturnal difficulty, exertional dyspnea, accessory muscle use, paradoxical breathing, ventilator dependent, poor cough/clearance of secretions
  6. Stage 1

    • Early disease
    • Mild focal weakness
    • Asymmetrical distribution
    • Symptoms of hand cramping and fasciculations

  7. Stage 2

    • Moderate weakness in groups of muscles
    • Some wasting (atrophy) of muscles
    • Modified independence with AD

  8. Stage 3

    • Severe weakness of specific muscles
    • Increasing fatigue
    • Mild to moderate functional limitations
    • Ambulatory

  9. Stage 4

    • Severe weakness and wasting of LEs
    • Mild weakness of UEs
    • Moderate assistance and AD required
    • WC user

  10. Stage 5

    • 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

  11. Stage 6

    • Bedridden
    • Dependent ADLs and FMS
    • Progressive respiratory distress 

  12. Examine

    • History (varied pattern of onset)
    • Vital signs
    • Respiratory function
    • CN function: especially lower cranial nerves (VII, IX, X, XI, XII)
    • Motor function (atrophy, weakness (symmetrical), cramping, twitching, spasticity, hyperreflexia)
    • Sensory function
    • Gait (timed walk)
    • Functional status
    • ALS Functional Rating Scale (ALSFRS)
  13. 
Functional Rating Scale (ALSFRS)

    • Assesses disease progression and function across 10 functional categories
    • 0 = loss of function
    • 4 = normal function
    • 40 = max score

  14. Medical Management

    • There is no effective treatment for this disease
    • Riluzole: a glutamate antagonist, may slow progression (especially with bulbar onset)

  15. PT Goals, Outcomes, and Interventions

    • Maintain respiratory function
    • Provide for nutritional needs
    • Prevent indirect impairments (PROM, positioning, skin care)
    • Provide moderate exercise program
    • Teach energy conservation activity
    • Maintain maximal functional independence (AD's, orthotic support, WC)
    • Symptomatic treatment of pains, spasms, spasticity

  16. PT Treatment = Maintain Respiratory Function

    • Airway clearance techniques
    • Cough facilitation
    • Breathing exercises
    • Chest stretching
    • Suctioning
    • Incentive spirometry
    • Long-term mechanical ventilation

  17. PT Treatment = Nutritional Needs

    • Management of dysphagia
    • May require nasogastric tube or PEG in later stages 

  18. PT Treatment = Exercise Precautions
    • Monitor fatigue levels closely
    • Avoid overwork injury
    • Avoid exercise if less than 1/3 of motor units are functioning
    • Limited positions with decreased pulmonary function

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