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NUR 213 Unit 6 - Myasthenia Gravis, ALS, & Guillain-Barre Syndrome
Terms in this set (20)
Autoimmune disorder that affects the body at the myoneuronal junction.
- The body breaks down the normal communication between nerve fibers and muscle cells.
- Involves motor but does not effect sensory.
- "Grave muscle weakness"
- NO CURE
Myasthenia Gravis - Pathophysiology
- Antibodies direct at the acetylcholine receptor sites and impair transmission of impulses across the myoneuronal junction.
- Fewer receptors are available for stimulation, causing voluntary muscle weakness.
- The thymus gland is believed to be the site of this antibody production.
- In adults with MG, the thymus gland remains large and has a tendency to form tumors.
Myasthenia Gravis - Clinical Manifestations
Muscle weakness that worsens after periods of activity and improves after periods of rest
- Weakened muscles that control eye and eyelid movement
- Diplopia (double vision)
- Ocular myasthenia
- Bulbar symptoms (weakness of the face and throat)
- Bland or masked expression
- Respiratory failure
- Unsteady gait
Myasthenia Gravis - Diagnostics
- Facial exam
- Ability to swallow
- Strength and tone of muscles
- Sense of touch
- Impaired eye movement
- CT or XR showing enlarged thymus
- Pulmonary function testing
Edrophonium Testing (Tensilon)
- Injectable medication that can improve facial weakness and ptosis within 5 minutes.
- Monitor cardiac and respiratory status when giving
- Have atropine at bedside when giving
- Ice pack is placed over eyes.
- Ptosis should improve within 2-5 minutes.
Myasthenia Gravis - Management
: (anticholinesterase) inhibits the breakdown of acetylcholine at the neuromuscular junction, improving transmission of the neural to muscle message. Increases the amount of available acetylcholine and increases muscle strength.
: suppresses production of harmful antibiodies
: Used if there is an inadequate response to steroids. Inhibits T-lymphocyte and B-lymphocyte production to reduce acetylcholine receptor antibody levels.
Exacerbation generalized by severe muscle, respiratory, and bulbar weakness.
- May result in respiratory failure.
- Adverse reaction to medications
Myasthenia Crisis - Symptoms
- Respiratory distress
- Prominent muscle weakness
Myasthenia Crisis - Management
- Nutritional support
- Use caution with sedatives
- Overmedication with cholinesterase inhibitors
- Stop cholinesterase inhibitors
- Nutritional support
Amyotrophic Lateral Sclerosis (ALS)
Progressive neurodegenerative disease, also known as Lou Gehrig's disease.
- Affects neurons in the brain and spinal cord.
- Starts in the hands or feet, and then spreads to other parts of the body.
- As the disease progresses, muscles grow weaker.
- Causes involuntary movements, difficulty walking, talking, chewing, swallowing, and speaking.
- Does not affect the bladder or senses
ALS - Pathophysiology
- As motor neuron cells die, the muscle fibers that they supply undergo atrophic changes.
- Neuronal degeneration may occur in both the upper and lower body.
- Muscle weakness usually begins in the cranial nerves, causing dysfunction of the face and neck.
- Some patients may even experience cognitive impairment.
- Eventually, respiratory function is impaired.
- Death commonly occurs as a result of infection, respiratory insufficiency, or aspiration.
ALS - Clinical Manifestations
Vary from person to person, and depend on the location of affected neurons.
- Fasciculations (muscle twitching)
- Lack of coordination/unsteady gait
- Difficulty speaking and swallowing
- Progressive muscle weakness of the arms and legs
- Difficulty performing ADLs
- Inappropriate crying, laughing, or yawning
- Overactive deep tendon reflexes
ALS - Management
- Goal: improve or maintain function, well-being, and quality of life.
- Riluzole: glutamine antagonist. Can aid in the transportation of signals between neurons and muscle fibers.
- Baclofen, dantrolene, diazepam
- Modafinil: used to treat fatigue
- Ventilator support
- Physical, occupational, speech
End of life care
Guillain Barre Syndrome
Rare syndrome in which the body's immune system attacks the peripheral nervous system.
- Also known as acute idiopathic polyneuritis
- May result in acute and rapid demyelination of the peripheral nervous system and some cranial nerves.
Guillain Barre Syndrome - Pathophysiology
A prior infection leads to an immune response
- The immune response cross-reacts with the peripheral nerve components
- Segmented demyelination of peripheral nerves occurs, preventing normal transmission of electrical impulses
- Sensorimotor nerve roots are affected; and autonomic transmission may be disrupted as well
Guillain Barre Syndrome - Types
: acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Most common type, causes weakness in the lower extremities that progresses upwards.
: strictly motor impairment with no alteration in sensation.
: descending, mostly affects the head and neck muscles.
: Miller-Fisher Syndrome (rare type)
Guillain Barre Syndrome - Common Causes
- Campylobacter (undercooked poultry)
- Influenza virus
- Epstein-Barr virus (causes mono)
- Zika virus
- Hepatitis A, B, C, and E
- Mycoplasma pneumonia
Guillain Barre Syndrome - Clinical Manifestations
Symmetrical muscle weakness
- Difficulty opening and closing eyes
- Difficulty swallowing and speaking
- Difficulty with balance and coordination
- Hyporeflexia / absent DTRs
- Pain (usually occurs from demyelination and nerve exposure)
- Bulbar weakness
- Throat, neck, and facial weakness
- Sluggish bowels and urinary retention
Guillain Barre Syndrome - Diagnostics
Presentation of symptoms
- Spinal tap / lumbar puncture (will contain increased protein and decreased WBCs)
- Nerve conduction studies
Guillain Barre Syndrome - Management
Respiratory function = Top priority
- May require mechanical ventilation
- Cardiac monitoring
- IVF for hypotension
- Alpha blockers for hypotension
- Passive ROM
- Physical therapy
- Q2h turns
- Decreased peristalsis, assess for ileus Consult dietician
- White boards for writing
- Alternative communication methods
- Cognition remains intact
- (effective treatment if initiated within 2 weeks of diagnosis)
- IVIG: high doses help to prevent myelin destruction
- Plasma exchange: removes the bad antibodies, helping to decrease time of illness
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