Amyotrophic Lateral Sclerosis (ALS)

Question 1

ALS Pathophysiology:

Answer 1

• Amyotrophic Lateral Sclerosis (ALS)
• “Lou Gehrig disease”
• Loss of *motor neurons in the anterior horn of the spinal cord and loss of motor nuclei of the lower brainstem
  — Prevents them from sending impulses to the muscles
• ALS is a progressive, degenerative neuromuscular disorder that involves death of motor neurons in the brain and spinal cord.
• Excess levels of glutamate in the brain and having served in the military

Question 2

ALS Causes and risk factors:

Answer 2

• Smoking
• Viral infections
• Autoimmune disease
• Genetic mutation/ hereditary dysfunctional immune response
• Environmental exposures to toxins
• Theory of cause: Over exposure to the neurotransmitter glutamate (excitatory neurotransmitter) results in cell injury and neuronal degeneration

Question 3

ALS S/S:

Answer 3

• Progressive weakness
• cramps, twitching (called fasciculation)
• lack of coordination
• Spasticity (deep tendon reflex brisk and overactive
• Muscles atrophy
• Fatigue
• loss of motor control
• If cranial nerves are involved it causes talking, swallowing, and breathing difficulties
• Pattern is not universal
• Intellectual functioning remains intact (most pts)

Question 4

ALS diagnostics & Laboratory Tests:

Answer 4

• History
• Various nerve and muscle tests (EMG)
  — Electromyography determines the severity of damage to the median nerve. A nerve conduction study assesses the nerve’s ability to send a signal along the nerve or to the muscle
• Magnetic resonance imaging (MRI) can show other conditions that may be causing the symptoms

Question 5

MRI Education:

Answer 5

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- no metal?

Question 6

ALS Nursing Interventions:

Answer 6

• Primarily supportive and educative
  — Adaptive devices help to maintain independence
  — Alternate means of communication need to be established as the ability to speak deteriorates
  — Spasticity (baclofan,valium)
• Hospitalization for complications
• Provide foods that are easy to swallow to prevent aspiration
• Assist with limb and trunk exercises to minimize spastic muslces.
• Maintain a safe environment
• Maintain fall precautions
• Provide tracheostomy care if client has a tracheostomy
• Initiate physical, occupational, speech, and psychological consults as prescribed
• Discuss end-of-life care

Question 7

What services do physical, occupational, and speech therapists provide to our patients?

Answer 7

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Question 8

ALS Medications:
Riluzole (Rilutek)

Answer 8

• Glutamate blocker-decreases the release of glutamate, thus minimizing damage to motor neurons
• Administer 2x a day on an empty stomach
• Neuro-protective (does not cure)
• Effectiveness is limited
• Side effects mimic disease
• Liver tests d/t toxicity

Question 9

ALS Medications:
To treat spasticity

Answer 9

• Baclofen (Lioresal) (muscle relaxer)
• Dantrolene Sodium (muscle relaxer)
• Diazepam (Valium)

Question 10

ALS Medications:
To treat fatigue

Answer 10

Modafinil (Provigil)