Neuromuscular Disorders Flashcards
What is amyotrophic lateral sclerosis (ALS)?
Muscle weakness caused by degeneration of motor neurons
Describe the pathophysiology of ALS (3)
- Begins with degeneration of LMNs (in spinal cord)
- Followed by degeneration of UMNs (in brain)
- As nerve cells die, muscles atrophy
Describe the progression of ALS
Upper extremities –> neck / throat –> trunk –> lower extremities
When are patients with ALS admitted? (2)
- Diagnosis
- Final stage of debilitation
The onset of death associated with ALS is within ______
5 years
Death from ALS is often due to ______
Pneumonia
What are the LMN manifestations of ALS? (3)
- Atrophy
- Fatigue
- Muscle weakness
What are the UMN manifestations of ALS? (2)
- Spastitity
- Hyperreflexia
What are the most common manifestations of ALS? (2)
- Dysphagia
- Dysarthria
What is dysarthria?
Slurred speech
Describe the primary goal of ALS treatment
Supportive care - no cure
What factor is required for the diagnosis of ALS?
Presence of anterior horn dysfunction in 3 limbs
What is the primary medication used for the treatment of ALS?
Minocycline - inhibits motor neuron death
Describe the nursing interventions associated with ALS (4)
- Assistive devices
- Conserve energy
- Prevent aspiration
- Encourage small, frequent, high-nutrient meals
What functions remain intact for a patient with ALS? (3)
- Cognition
- Sensation
- Bowel / bladder control
What are the priority assessments associated with ALS? (2)
- Respiratory function
- Swallowing function
What is myasthenia gravis (MG)?
Weakness / fatigue of voluntary muscles
Describe the pathophysiology of MG (3)
- Antibodies act against acetylcholine receptors
- Decreased acetylcholine
- Increased cholinesterase
Acetylcholine is responsible for ______
Muscle contraction
Cholinesterase is responsible for ______
Breakdown of acetylcholine
What are the manifestations of MG? (4)
- Asymmetrical facial drooping
- Downward turn of the mouth
- Ptosis
- Diplopia
What is ptosis?
Eyelid drooping
What is diplopia?
Double vision
What functions are most affected by MG? (4)
- Facial expressions
- Chewing
- Swallowing
- Speech
What is the primary diagnostic test used for the diagnosis of MG?
Anticholinesterase testing - tensilon
Describe the MOA of tensilon
Increases acetylcholine at neuromuscular junctions by blocking cholinesterase
How is tensilon used for the diagnosis of MG? (2)
- Improvement of muscle strength - indication of MG diagnosis
- No improvement of muscle strength - rules out MG diagnosis
What medications are used for the treatment of MG? (3)
- Prostigmin - improves muscle strength
- Mestinon - improves muscle strength
- IV Atropine - counteracts cholinergic reactions
When administering MG medications, it is important to monitor for ______
Cholinergic side effects
What are some examples of cholinergic side effects? (3)
- Excessive salivation
- Sweating
- Diarrhea
Describe the primary nursing consideration associated with MG medications
Must be given at exact same time everyday
What are the complications associated with MG? (2)
- Myasthenic crisis
- Cholinergic crisis