Amyotrophic Lateral Sclerosis (ALS)

Question 1

What is ALS?

Answer 1

Rare progressive Neuromuscular disorder *NOT AUTOIMMUNE *
Loss of motor neurons
Neurological degeneration affects both upper and lower neurons
Lou Gehrig
Stephen Hawking
Jason Becker

Question 2

ALS etiology

Answer 2

book diagnosed 40-60 yrs Men >Women
Motor neuron in the brainstem and spinal cord gradually degenerate for unknown reasons.
Dead motor neurons cannot produce or transport signals to muscles.
Electrical and chemical messages originating in the brain DO NOT reach the muscles to activate them.

Question 3

Clinical manifestations of ALS

Answer 3

• Fatigue *defining symptoms
• Progressive Muscle twitching * defining symptoms
• Lack of coordination * defining symptoms
• spasticity
• difficulty talking
• difficulty swallowing

Question 4

Complications of ALS

Answer 4

• 100% cognitively intact
• Respiratory Function compromised
• Death results from respiratory tract infection 2/2 compromised respiratory function*

Question 5

Diagnosing ALS

Answer 5

• based on S/S
• no lab test or clinical test
• Muscle biopsy
• MRI
• EMG

Question 6

Pharmacological management in ALS

Answer 6

• Dantrolene Sodium (Dantrium): #1 go- to drug
• Baclofen: muscle spasms #1 go-to drug along w/ Dantrium
• Riluzole (Rilutek): slows progression by decreasing gluconate (excitatory neurotransmitter) in the brain.
• Diazepam (Valium)

Question 7

Important to do with ALS patients

Answer 7

1. Facilitate communication
2. Reduce risk of aspiration
3. Decrease pain of muscle weakness
4. Decrease pain from injury
5. Altered bowel function