GF7 GF/LURN: Neuro ALS & GBS

Question 1

What is Amyotrophic Lateral Sclerosis (ALS)?

Answer 1

• ALS also called Lou Gehrig’s disease presents as deterioration of motor neurons in the brain & spinal cord, resulting in progressive TOTAL BODY paralysis
• Eventually clients die in 3 - 5 years from Respiratory Failure
• Cognitive function not impaired

Question 2

What is the patho of ALS?

Answer 2

• Exact cause is unknown
• A mutate (SODI) gene produces a defective protein that is toxic to motor neurons and found in 20% of inherited cases
• Excess glutamate oxidative and free radical damage, mitochondrial dysfunction and activation of the cell death pathways
• Gradual deterioration of the upper and lower motor neurons leads to loss of voluntary movement and muscle control

Question 3

What are the risk factors for ALS?

Answer 3

• White
• > 40yrs
• Family hx

Question 4

What are the s/s of ALS?

Answer 4

• Progressive muscle & limb weakness
• Dyspnea, increased secretions
• Dysphagia
• Respiratory paralysis (w/in 3-5yrs)
• Constipation

Question 5

How is ALS diagnosed?

Answer 5

By clinical symptoms and ruling out other neurologic diseases

Question 6

What is the tx for ALS?

Answer 6

No cure

Rilutek/Riluzole slows deterioration of motor neurons by protecting the nerve cells from overexposure to glutamate

Question 7

What is the nursing care for ALS?

Answer 7

• Maintain patent airway
• Monitor for infection (fever 100.3°), pneumonia, respiratory failure
• Rhonchi = infection w/ a lot of mucus
  
  • do not confuse with crackles which = pulmonary edema
  • do not confuse with wheezing which = asthma
• Coordinate w/ palliative team

Question 8

True or False

90% of ALS cases have a family hx.

Answer 8

True

Question 9

What is GB Syndrome?

Answer 9

An autoimmune disorder affecting the peripheral nervous system that causes a sudden onset of weakness and paralysis

Question 10

What is the patho of GB?

Answer 10

Usually follows a respiratory or GI viral infection

Leading to autoimmune destruction of the myelin sheath (made by schwann cells) and axons in motor and sensory nerves

Question 11

How does GB progress?

Answer 11

• Begins in the lower extremities and ascends bilaterally w/:
  
  • Weakness
  • Ataxia (group of disorders that affect co-ordination, balance and speech)
  • Bilateral paresthesia progressing to paralysis

Question 12

What are the s/s of GB?

Answer 12

• Ascending symmetrical muscle weakness
• Lower extremity weakness
• Absent deep-tendon reflexes
• Neuromuscular respiratory failure

Question 13

What are the early signs of respiratory failure with GBS?

Answer 13

• Bulbar weakness
  
  • difficulty swallowing, weak jaw and facial muscles, progressive loss of speech, and weakening of the tongue
• Inability to cough
• Inability to lift head or eyebrows
• Shallow respirations
• Dyspnea and hypoxia
• Autonomic dysfunction (irregular HR/BP, abnormal diaphoresis, changes in pupillary reaction)

Question 14

What is the most important nursing interventions for a GB pt?

Answer 14

Get intubation setup bedside w/ ventilator

Monitor for aspiration, pneumonia, respiratory failure

Question 15

How long does it take for GB to progress?

Answer 15

Can be a few hours to a few days, but typically within 2 weeks and no longer than 4 weeks

Question 16

What dx tests are done for GB?

Answer 16

• MRI
• Spinal tap: w/ elevated protein level and WBCs WNL
• Electromyography (EMG) for abnormal nerve conduction

Question 17

Can a full recovery be made from GB?

Answer 17

• Yes, as the schwann cells can replace the degraded myelin
• Most people eventually make a full recovery from Guillain-Barré syndrome, but this can sometimes take a long time and around 1 in 5 people have long-term problems.
• The vast majority of people recover within a year.
• A few people may have symptoms again years later, but this is rare.

Question 18

What is the tx for GB?

Answer 18

IViG

Plasmapheresis

Question 19

What is IGG therapy?

Answer 19

• Immunoglobulin replacement therapy is a treatment given to boost the IgG antibody levels when they are low.
• This treatment can strengthen the immune system and help immune deficient patients fight off infections.

Question 20

What is plasmapheresis?

Answer 20

• a method of removing blood plasma from the body by withdrawing blood, separating it into plasma and cells, and transfusing the cells back into the bloodstream.
• It is performed especially to remove antibodies in treating autoimmune conditions

Question 21

Elevated protein in CSF is indicative of…

Answer 21

Autoimmune disease

Question 22

True or False

PT/OT treatment will be needed to restore previous muscular function w/ GB.

Answer 22

True