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Myasthenia Gravis

An autoimmune neurologic
         disorder



            By Lexi Gray & Mary Kacic
Background

 Myasthenia gravis means “grave muscle
  weakness”
 It is an autoimmune disease affecting the
  myoneural junction
 About 60,000 people in the U.S. have this
  disease
 Women affected more frequently than men, at
  around 20-40 years of age. Men develop it
  later, at around 60-70 years of age.
Symptoms
 Causes varying degrees of skeletal muscle
  weakness
 Hallmark of the disease: muscle weakness
  increases during periods of activity and improves
  during rest.
 Areas that MAY be affected: eyes/eyelid
  movement, facial expression, chewing, talking,
  swallowing
Pathophysiology
 Normal communication between the nerve and muscle
  is interrupted at the neuromuscular junction (where
  nerve cells meet with the muscles they control)
 Normally, nerve endings release acetylcholine which
  travels through the NMJ and binds to receptors, causing
  muscle contraction.
 In myasthenia gravis, these receptors are blocked or
  destroyed
Pathophysiology
 Autoimmune disease: the antibodies that attack
  or block the acetylcholine receptors are produced
  by the body’s own immune system
 Thymic hyperplasia or a thymic tumor is present
  in 80% of people with myasthenia gravis
Assessment

 Onset of the disorder may be sudden
 Many times, symptoms are not immediately
  recognized as being related to myasthenia gravis
 In most cases, weakness of the eye muscles is
  noticed first.
Assessment - Severe Symptoms
  Ptosis – drooping of one or both eyelids
  Diplopia – blurred or double vision
  Bulbar symptoms – weakness of muscles of the
   face & throat
  Bland facial expression
  Difficulty swallowing – increased risk of choking &
   aspiration
  Dysarthia – impaired speech
  Dysphonia – voice impairment
  Weakness In arms, hands, legs, neck
  Generalized weakness also effects intercostal
   muscles, resulting in decreasing vital capacity and
   respiratory failure.
Confirming the Diagnosis
 Acetylcholinesterase Inhibitor Test
   Confirms the diagnosis of myasthenia gravis. During
    this test, the breakdown of acetylcholine is stopped.
    As a result, acetylcholine is able to accumulate at the
    receptor sites.
   For this test, edrophoniumchloride is administered IV.
    Facial muscle weakness and ptosis will resolve about
    30 second after the drug is administered, if the patient
    is positive for a myasthenia gravis diagnosis. This
    relief lasts only 5 minutes, so it is not considered a
    treatment.
   After the drug is administered, the patient's serum is
    then tested for acetylcholine receptor antibodies,
    which would also be consistent with the diagnosis.
   In some cases, patients with myasthenia gravis may
    have an enlarged thymus gland.
Nursing Diagnosis #1
 Risk for aspiration R/T difficulty swallowing,
  weakness of bulbar muscles
 Overall goal: No aspiration will occur
 Interventions
   Give meals with anticholinesterase meds to inhibit
    breakdown of acetylcholine and increase its
    concentration at the NMJ
   Raise the HOB to semi-fowler’s position, which will
    ensure upper airway patency.
   Give food with a pudding-like consistency, which
    can be more easily swallowed.
Nursing Diagnosis #2
 Deficient knowledge R/T drug therapy, potential
  for crisis (myasthenic or cholinergic), and self-
  care management
 Overall goal: Patient will demonstrate knowledge
  of medication management, importance of rest,
  coping strategies, and prevention/management of
  complications
 Interventions
   Teach patient about the actions of meds, and
    importance of regimen. The patient will verbalize
    the consequences delaying med intake, including
    S&S of myasthenic and cholinergic crisis.
   Teach strategies for patient to conserve their
    energy. The patient will develop coping strategies to
Treatment of myasthenia gravis
 Pharmacologic Treatment
  First-line med is pyridostrigmine bromide
   (Mestinon), an anticholinesterase that inhibits the
   breakdown, thus improving skeletal muscle
   contraction. Side effects can include fasciculations,
   abdominal pain, diarrhea.
  Immunosuppressive Therapy: Corticosteroids
   decrease the amount of antibody production.
   Cytotoxic meds (Azathioprine) inhibits production of
   T-and B-cells, and effects may not be seen for 3-12
   months. Hepatotoxicity is a risk of using cytotoxic
   meds.
  Some common medications exacerbate the
   symptoms of myasthenia gravis, including:
Possible Side Effects of
Anticholinesterase Meds
 Central          Respiratory/           Skeletal Muscles
 Nervous System   Cardiovascular
 Irritability     Bronchial relaxation   Fasciculations
 Anxiety          Increased bronchial    Spasms
                  secretions
 Insomnia         Tachycardia            Weakness
 Headache         Hypotension
                                         Genitourinary
 Dysarthia                               Frequency
                  Gastrointestinal
 Syncope          Abdominal cramps       Urgency
 Seizures         Nausea,vomiting and
                  diarrhea               Integumentary
 Coma             Anorexia               Rash
 Diaphoresis      Increased Salivation   Flushing
Treatments
 Plasmatheresis
   This procedure removes the patient’s plasma and
   plasma components through a centrally placed
   catheter. Blood cells are separated from antibody-
   containing plasma. The blood cells are then mixed
   with plasma substitute and reinfused. Provides
   temporary treatment for severe symptoms.
 Intravenous immune globulin
    Treats exacerbations of myasthenia gravis
    temporarily. This procedure does not work as
    quickly as plasmatheresis.

 These are treatments for myasthenia gravis, not
  cures.
Treatments
 Surgical Treatments
  Thymectomy: Surgical removal of the thymus
   gland, which may result in the production of
   antigen-specific immunosuppression, which
   results in clinical improvement. Results vary;
   patient may have partial or complete
   remission, or no remission at all.
    Nursing considerations for the patient who
    received a thymectomy include monitoring
    respiratory function and mechanical ventilation.
Myasthenia (or cholinergic) Crisis
  Exacerbation of the disease process. Signs &
     symptoms include muscle/bulbar weakness.
    Causes for myasthenia crisis may include a
     respiratory infection, pregnancy, or medications.
    Primary management is focused on maintaining the
     airway. The nurse assesses respiratory rate, depth,
     breath sounds, and pulmonary function.
     Endotracheal intubation and mechanical ventilation
     may be necessary.
    Assess arterial blood gases, I&O, daily weight.
    Avoid sedatives and tranquilizers.
    Bradycardia and respiratory distress (emergency
     situations) are treated with atropine.
References
Doenges, M., Moorhouse, M., Murr, A. (2010).
 Nurse’s Pocket Guide: Diagnoses, Prioritized
 Interventions, and Rationales (12th ed.).
 Philadelphia: F.A. Davis. ISBN: 0803622341.
Smeltzer, S. C., Bare, B. G., Hinkle, J. L. &
 Cheever, K. H. (eds.) (2008). Brunner &
 Suddarth's textbook of medical-surgical nursing
 (11th ed.). Philadelphia: Lippincott Williams &
 Wilkins.

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  • 1. Myasthenia Gravis An autoimmune neurologic disorder By Lexi Gray & Mary Kacic
  • 2. Background  Myasthenia gravis means “grave muscle weakness”  It is an autoimmune disease affecting the myoneural junction  About 60,000 people in the U.S. have this disease  Women affected more frequently than men, at around 20-40 years of age. Men develop it later, at around 60-70 years of age.
  • 3. Symptoms  Causes varying degrees of skeletal muscle weakness  Hallmark of the disease: muscle weakness increases during periods of activity and improves during rest.  Areas that MAY be affected: eyes/eyelid movement, facial expression, chewing, talking, swallowing
  • 4. Pathophysiology  Normal communication between the nerve and muscle is interrupted at the neuromuscular junction (where nerve cells meet with the muscles they control)  Normally, nerve endings release acetylcholine which travels through the NMJ and binds to receptors, causing muscle contraction.  In myasthenia gravis, these receptors are blocked or destroyed
  • 5. Pathophysiology  Autoimmune disease: the antibodies that attack or block the acetylcholine receptors are produced by the body’s own immune system  Thymic hyperplasia or a thymic tumor is present in 80% of people with myasthenia gravis
  • 6. Assessment  Onset of the disorder may be sudden  Many times, symptoms are not immediately recognized as being related to myasthenia gravis  In most cases, weakness of the eye muscles is noticed first.
  • 7. Assessment - Severe Symptoms  Ptosis – drooping of one or both eyelids  Diplopia – blurred or double vision  Bulbar symptoms – weakness of muscles of the face & throat  Bland facial expression  Difficulty swallowing – increased risk of choking & aspiration  Dysarthia – impaired speech  Dysphonia – voice impairment  Weakness In arms, hands, legs, neck  Generalized weakness also effects intercostal muscles, resulting in decreasing vital capacity and respiratory failure.
  • 8. Confirming the Diagnosis  Acetylcholinesterase Inhibitor Test  Confirms the diagnosis of myasthenia gravis. During this test, the breakdown of acetylcholine is stopped. As a result, acetylcholine is able to accumulate at the receptor sites.  For this test, edrophoniumchloride is administered IV. Facial muscle weakness and ptosis will resolve about 30 second after the drug is administered, if the patient is positive for a myasthenia gravis diagnosis. This relief lasts only 5 minutes, so it is not considered a treatment.  After the drug is administered, the patient's serum is then tested for acetylcholine receptor antibodies, which would also be consistent with the diagnosis.  In some cases, patients with myasthenia gravis may have an enlarged thymus gland.
  • 9. Nursing Diagnosis #1  Risk for aspiration R/T difficulty swallowing, weakness of bulbar muscles  Overall goal: No aspiration will occur  Interventions  Give meals with anticholinesterase meds to inhibit breakdown of acetylcholine and increase its concentration at the NMJ  Raise the HOB to semi-fowler’s position, which will ensure upper airway patency.  Give food with a pudding-like consistency, which can be more easily swallowed.
  • 10. Nursing Diagnosis #2  Deficient knowledge R/T drug therapy, potential for crisis (myasthenic or cholinergic), and self- care management  Overall goal: Patient will demonstrate knowledge of medication management, importance of rest, coping strategies, and prevention/management of complications  Interventions  Teach patient about the actions of meds, and importance of regimen. The patient will verbalize the consequences delaying med intake, including S&S of myasthenic and cholinergic crisis.  Teach strategies for patient to conserve their energy. The patient will develop coping strategies to
  • 11. Treatment of myasthenia gravis  Pharmacologic Treatment  First-line med is pyridostrigmine bromide (Mestinon), an anticholinesterase that inhibits the breakdown, thus improving skeletal muscle contraction. Side effects can include fasciculations, abdominal pain, diarrhea.  Immunosuppressive Therapy: Corticosteroids decrease the amount of antibody production. Cytotoxic meds (Azathioprine) inhibits production of T-and B-cells, and effects may not be seen for 3-12 months. Hepatotoxicity is a risk of using cytotoxic meds.  Some common medications exacerbate the symptoms of myasthenia gravis, including:
  • 12. Possible Side Effects of Anticholinesterase Meds Central Respiratory/ Skeletal Muscles Nervous System Cardiovascular Irritability Bronchial relaxation Fasciculations Anxiety Increased bronchial Spasms secretions Insomnia Tachycardia Weakness Headache Hypotension Genitourinary Dysarthia Frequency Gastrointestinal Syncope Abdominal cramps Urgency Seizures Nausea,vomiting and diarrhea Integumentary Coma Anorexia Rash Diaphoresis Increased Salivation Flushing
  • 13. Treatments  Plasmatheresis  This procedure removes the patient’s plasma and plasma components through a centrally placed catheter. Blood cells are separated from antibody- containing plasma. The blood cells are then mixed with plasma substitute and reinfused. Provides temporary treatment for severe symptoms.  Intravenous immune globulin  Treats exacerbations of myasthenia gravis temporarily. This procedure does not work as quickly as plasmatheresis. These are treatments for myasthenia gravis, not cures.
  • 14. Treatments  Surgical Treatments  Thymectomy: Surgical removal of the thymus gland, which may result in the production of antigen-specific immunosuppression, which results in clinical improvement. Results vary; patient may have partial or complete remission, or no remission at all.  Nursing considerations for the patient who received a thymectomy include monitoring respiratory function and mechanical ventilation.
  • 15. Myasthenia (or cholinergic) Crisis  Exacerbation of the disease process. Signs & symptoms include muscle/bulbar weakness.  Causes for myasthenia crisis may include a respiratory infection, pregnancy, or medications.  Primary management is focused on maintaining the airway. The nurse assesses respiratory rate, depth, breath sounds, and pulmonary function. Endotracheal intubation and mechanical ventilation may be necessary.  Assess arterial blood gases, I&O, daily weight.  Avoid sedatives and tranquilizers.  Bradycardia and respiratory distress (emergency situations) are treated with atropine.
  • 16. References Doenges, M., Moorhouse, M., Murr, A. (2010). Nurse’s Pocket Guide: Diagnoses, Prioritized Interventions, and Rationales (12th ed.). Philadelphia: F.A. Davis. ISBN: 0803622341. Smeltzer, S. C., Bare, B. G., Hinkle, J. L. & Cheever, K. H. (eds.) (2008). Brunner & Suddarth's textbook of medical-surgical nursing (11th ed.). Philadelphia: Lippincott Williams & Wilkins.