Amyotrophic Lateral Sclerosis CAT Flashcards
1
Q
What is ALS?
A
- A chronic degenerative disease that produces both upper and lower motor neuron impairments
- Symptoms are caused by demyelination, axonal swelling, and atrophy in the cerebral cortex, premotor areas, sensory cortex, and temporal cortex
2
Q
Degeneration and demyelination will cause what phenomena in patients?
A
- Denervation of muscle fibers
- Muscle atrophy
- Weakness
3
Q
What causes ALS?
A
- Etiology is unknown
- Theories include genetic inheritance, a slow acting virus, metabolic disturbance, and toxicity of lead and aluminum
4
Q
What lower motor neuron signs will be seen?
A
- Asymmetric muscle weakness
- Cramping
- Atrophy (usually in the hands)
- Weakness follows a distal to proximal path
5
Q
What upper motor neuron signs will be seen?
A
- Caused by loss of inhibition of the muscle
- Incoordination of movement
- Spasticity
- Clonus
- Babinski reflex
6
Q
Signs of bulbar involvement
A
- Dysarthria
- Dysphagia
- Emotional lability
7
Q
Clinical presentation of ALS
A
- Fatigue
- Oral motor impairment
- Fasciculations
- Spasticity
- Motor paralysis
- Eventual respiratory paralysis
8
Q
What role will lab values or imaging play in managing ALS?
A
- EMG to assess fibrillation and fasciculations
- Muscle biopsy to verify LMN involvement instead of muscle disease
- Spinal tap may reveal a higher protein content in some patients
- CT scan will appear normal until late stage
9
Q
What information is needed to diagnose ALS?
A
- Heavy reliance on symptoms
- Rule out other diagnoses
10
Q
What neurological functions will be preserved with ALS?
A
- Sensation
- Eye movement
- Bowel and bladder function
11
Q
What functions are lost with ALS?
A
- Distal –> proximal strength
- Paralysis of vocal cords
- Swallowing impairment
- Contractures
- Ulcers
- Breathing difficulty
12
Q
How is ALS managed?
A
- Pharmacological management: riluzole (rilutek)
- Anticholinergic, antispasticity, and antidepressent medication
13
Q
Role of physical therapy with ALS
A
- Maintaining quality of life
- Low-level exercise
- ROM
- Mobility training
- Assistive devices
- WC prescription
- Bronchial hygiene
- Energy conservation
14
Q
How is exercise prescribed for patients with ALS?
A
Low-level as long as the patient does not exercise to fatigue
15
Q
Average course of ALS?
A
- 2-5 years
- About 1/4 live longer than 5 years
- Pts diagnosed before 50 years old usually live longer
