Amyotrophic Lateral Sclerosis (ALS)

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Amyotrophic Lateral Sclerosis (ALS)
2014-05-03 18:36:18

review of ALS lecture 4/23
Show Answers:

  1. What type of disease is ALS?
    motor neuron disease
  2. Motor Neuron Disease
    • progressive bulbar palsy (upper and lower motor neurons)
    • --life expectancy 6 mo to 3 years from diagnosis
    • progressive/spinal muscular atrophy (lower motor neurons of spinal cord and brain stem)
    • --live > 5 years
    • Primary lateral sclerosis (upper motor neurons)
    • --may have normal life expectancy
    • ALS (upper and lower neurons)
    • --most common
    • --life expectancy 2-5 years from diagnosis
  3. ALS description:
    • irreversible, progressive disorder of the nervous system
    • degeneration of both UMN and LMN
    • neurons in the motor cortex and brainstem and anterior horn cells in the spinal cord stop relaying messages to the mm (weakness, atrophy, and fasciculations)
    • loss of voluntary movement, then death
    • the chest and diaphragm mm become involved and the person is no longer able to breathe
    • respiratory failure is leading cause of death
  4. Affect men or women more?
  5. Age of onset:
    55-75, peak 62
  6. amyotrophy:
    atrophy of the muscle fibers as their corresponding anterior horn cells degenerate
  7. Lateral sclerosis:
    hardening of the anterior and lateral columns of the spinal cord as the motor neurons degenerate and are replaced by fibrous astrocytes (gliosis)
  8. Disease Course:
    • onset to ventilator dependence of death is 2-4 years
    • after ventilator mean life expectancy is approx. 5 yrs but can exceed 10
    • skin integrity
  9. Despite the progressive nature of the disease, what functions remain normal?
    • cognition
    • occulomotor
    • sensation
    • bowel/bladder
  10. What is research of ALS focused on?
    finding the source of neuron degeneration and stopping it
  11. Clinical manifestations in LMN:
    • asymmetrical weakness
    • cramping w/ voluntary movement in the early morning
    • fasciculations
    • extensor mm become weaker than flexor mm; hand
  12. Clinical Manifestations UMN:
    • positive babinski
    • positive hoffman's sign
    • hyperreflexivity
  13. Are both LMN and UMN symptoms present?
  14. Pharmacological Management for symptoms:
    • baclofen for spasticity or anticonvulsants (gabapentin or neurotin)
    • NSAIDs for pain
    • Benzodiazepines for severe fasciculations
    • antioxidant rich food may slow impact of ALS
  15. Pharmacological management for disease mechanism:
    • rilusole/rilutek -- increase time until trach or death
    • no improvement of muscle strength or neurological fxn
  16. Indicators for therapy:
    • fatigue
    • spasticity
    • functional losses are noted when isometric strength declines
    • progressive weakness of scapulohumeral musculature and subluxation
    • contractures (need stretching)
    • unable to shift weight for pressure relief (recline schedule)
    • loss of diaphragm (abs to help)
  17. PT
    • slow stretching for spasticity and cramping
    • daily ROM taught to caregivers
    • safe and efficient transfers
    • initially, assistive device for walking w/ AFO for foot drop
    • WC w/ reassess as disease progresses
    • sub-maximal exercise for those w/ slow progression of disease
  18. OT
    • energy conservation
    • assessment and intervention for dysphagia
    • adaptive equipment for meaningful activities
    • -universal cuff for decreased grip
    • -splint or brace into 20-25 degrees of extension to increase grip strength
    • -balanced forearm orthosis
    • -slings/taping to reduce pain due to shoulder girdle weakness
    • -environment control units (eye movements)
  19. Most importantly:
    • understand the wants and needs of the person
    • anticipate the progressive nature of this disease
    • problem solve w/ them to determine how you can best assist them; including making recommendations for AT
    • understand and appreciate the impact of the environment to help them remain as functional for as long as possible
    • work w/ caregiver and other providers