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Myasthenia Gravis

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Myasthenia Gravis Grave Muscle Weakness Myasthenia Gravis Autoimmune disease affecting the neuromuscular junction Not a brain disorder brain functions ... – PowerPoint PPT presentation

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Title: Myasthenia Gravis


1
Myasthenia Gravis
  • Grave Muscle Weakness

2
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3
Myasthenia Gravis
  • Autoimmune disease affecting the neuromuscular
    junction
  • Not a brain disorder brain functions normally
  • Characterized by fluctuating muscle weakness and
    fatigability
  • Disease may be generalized or ocular specific

4
Pathophysiology
  • Antibodies attack Acetylcholine (ACH) receptors
    at the motor end plate
  • Decreased number of (ACH) receptor sites at
    neuromuscular junction
  • Prevents neurotransmitter (ACH) from attaching
    and stimulating muscle contraction
  • Resulting in loss of muscle strength

5
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6
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7
Causes
  • No single cause has been identified
  • Abnormal thymus tissue found in most patients
    with MG
  • Thymic tumors found in 15 of patients
  • Virus infections have been found in some cases
    and are a suspected cause

8
Incidence
  • Occurs in all races
  • MG affects 14 per 100,000 people in the United
    States
  • Can affect any age group
  • Women peak incidence 20's to 30's
  • Men peak incidence 50's to 60's
  • Three times more common in women than men
  • Juvenile onset or over the age of 70 onset is
    extremely rare

9
Genetic Factors
  • Myasthenia Gravis is not a genetically inherited
    disease
  • Some families appear to carry a gene that
    increases the risk for developing the disease
  • No specific gene has been identified and there
    are no tests for genetic screening

10
Diagnosis
  • Edrophonium test (Tensilon)
  • Blood analysis
  • Repetitive nerve stimulation
  • Single-fiber electromyography (EMG)
  • Imaging scans

11
Tensilon Test
  • Injection may result in a sudden, although
    temporary, improvement in muscle strength an
    indication of myasthenia gravis. Acts to block an
    enzyme that breaks down acetylcholine, the
    chemical that transmits signals from nerve
    endings to muscle receptor sites.

12
Signs and Symptoms
  • Affects any of the muscles that you control
    voluntarily, certain muscle groups are more
    commonly affected than others
  • Eye, face, throat, neck, limb muscles
  • Difficulty speaking (dysarthria)
  • Difficulty swallowing (dysphagia),
  • Drooping eyelids (ptosis)
  • Double vision (diplopia)
  • Nasal-sounding speech and weak neck muscles that
    give the head a tendency to fall forward or
    backward. 

13
Signs and Symptoms
  • Symptoms tend to progress over time, usually
    reaching their worst within a few years after the
    onset of the disease
  • Worsening muscle weakness with repeat activity

14
Exacerbation Trigger Factors
  • Infection
  • Stress
  • Fatigue
  • Cathartics (laxatives)
  • Heat (sauna, hot tubs, sunbathing)

15
Diet/Nutrition
  • Eat small meals and snacks five to six times a
    day
  • Avoid using low fat or diet products when
    possible
  • Avoid eating lemons or tonic water
  • Eat warm rather than hot food
  • Runny or puree diet when swallowing is difficult
  • Alternate sips of liquid to avoid food from
    sticking
  • AVOID eating chewy or dry crumbly foods


16
Medications/Treatment
  • Immunosuppressive Therapy
  • Prednisone
  • Azathioprine
  • Acetylcholinesterase Inhibitors
  • First line of therapy
  • Neostigmine bromide (Pyridostigmine)
  • Edrophonium chloride (Tensilon)

17
Medications/Treatment
  • Plasmapheresis
  • Immunoglobulin Therapy
  • Thymectomy

18
Myasthenic Crisis VS. Cholinergic Crisis
  • Myasthenic Crisis
  • Under medication
  • Increased HR/BP/RR
  • Bowel and bladder incontinence
  • Decreased urine output
  • Absent cough and swallow reflex
  • May need mechanical ventilation
  • Temporary improvement of symptoms with
    administration of Tensilon
  • Cholinergic Crisis
  • Overmedication
  • Decreased BP
  • Abd cramps
  • N/V, Diarrhea
  • Blurred vision
  • Pallor
  • Facial muscle twitching
  • Constriction of pupils
  • Tensilon has no effect
  • Symptoms improve with administration of
    anticholinergics (Atropine)

19
Nursing Interventions
  • Maintain patent airway
  • Assess swallowing to prevent aspiration
  • Keep appropriate equipment available at the
    bedside Oxygen, suction, Ambu bag, endotracheal
    intubation
  • Promote energy conservation measures
  • Consult Physical Therapy for medical equipment
    needs (i.e.walkers, wheelchairs)

20
Nursing Interventions
  • Consult OT for assistive devices to facilitate
    ADLs
  • Consult with speech and language therapist if
    weakening facial muscles impact communication
  • Monitor I/O, serum albumin levels, and daily
    weights
  • Know the signs and symptoms of both Myasthenic
    Crisis and Cholinergic Crisis
  • Administer Medications
  • Immunosuppressants Prednisone, Imuran
  • Cholinesterase Inhibitors Pyridostigmine

21
Patient Teaching
  • Teach patient/family disease process,
    complications, and treatments
  • Teach patient about their medications uses dosage
    etc
  • Teach medications to use with caution d/t muscle
    exacerbation
  • Beta blockers, calcium channel blockers, quinine,
    quinidine, procainamide, some antibiotics,
    neuromuscular blocking agents
  • Avoid certain medications
  • D-penicillinamine, A-interferon, botulinum toxin

22
Patient Teaching
  • Teach s/sx of both Myasthenic Crisis and
    Cholinergic Crisis
  • Help patient plan daily activity to coincide with
    energy peaks
  • Stress need for rest periods
  • Explain that exacerbations, remissions, and daily
    fluctuations are common
  • Avoid strenuous exercise, stress, infection,
    exposure to hot or cold temperatures
  • Teach patient to wear medic-alert bracelet
  • Provide Info on Support groups
  • www.myasthenia.org

23
Prognosis
  • Chronic disease with periods of exacerbation and
    sometimes remissions
  • Disease course is highly variable
  • Symptoms respond well to treatment and in most
    cases the patient can live a normal or nearly
    normal life
  • Ocular Myasthenia has the best prognosis
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