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MYASTHENIA
GRAVIS
By: Hiren K Gehloth
F.Y.M.Sc Nursing
Govt.College Of
Nursing,Abad(GINERA)
INTRODUCTION
 Myasthenia gravis means “grave muscle
weakness’ or “weakness of the voluntary or
striated muscles of the bo...
 Myasthenia gravis (MG) is a relatively rare autoimmune
disorder in which antibodies form against nicotinic
acetylcholine...
 The ocular and bulbar muscles are affected most
commonly and most severely, but most patients also
develop some degree o...
DEFINITION
 “Myasthenia Gravis is a auto immune disorder affecting
the myoneural junction, characterized by varying degre...
CAUSES AND RISK FACTORS
 MG is idiopathic in most patients. Although the main cause
behind its development remains specul...
 Thymic abnormalities are
common: Of patients with
MG, 75% have thymic
disease, 85% have thymic
hyperplasia, and 10–15% h...
PATHOPHYSIOLOGY
TYPES OF MYASTHENIA GRAVIS
→ Congenital myasthenia gravis
Congenital myasthenia gravis is defined by its cause: a
genetic ...
→Ocular myasthenia Gravis
 In ocular myasthenia gravis, the muscles that move the eyes and
control the eyelids are weaken...
 While many patients with other forms of myasthenia gravis
first experience symptoms in the muscles around the eyes as
we...
 In approximately 10 percent of these patients, muscle
weakness spreads to the respiratory muscles or muscles of
the thro...
→ Juvenile myasthenia gravis
 In juvenile myasthenia gravis, symptoms begin before the
onset of puberty.
 Juvenile myast...
CLINICAL MANIFESTAION
 Drooping of one or both eye lids (ptosis)
 Blurred or double vision
 Breathing difficulty becaus...
•Drooping head
•Facial paralysis or weakness of the
facial muscle
•Mask- like facial expression
•Easy fatigability
•Hoarse...
DIAGNOSTICEVALUATION
 A physical and neurological examination.
 A blood test.
 An Edrophonium test.
This test uses inje...
BEFORE AFTER
 Electrodiagnostics. Diagnostic tests include repetitive
nerve stimulation, which repeatedly stimulates a person’s
nerves...
 Pulmonary function testing. Measuring breathing strength
can help predict if respiration may fail and lead to a
myasthen...
MANAGEMENT
Thymectomy- This operation to remove the thymus gland
(which often is abnormal in individuals with myasthenia
g...
 A recent NINDS-funded study found that thymectomy is
beneficial both for people with thymoma and those with no
evidence ...
 Immunosuppressive drugs. These drugs improve muscle
strength by suppressing the production of abnormal
antibodies. They ...
 Plasmapheresis is a procedure using a machine to remove
harmful antibodies in plasma and replace them with good
plasma o...
 Intravenous immunoglobulin is a highly concentrated
injection of antibodies pooled from many healthy donors
that tempora...
COMPLICATION
NURSING MANAGEMENT
 Administer cholinesterase inhibitors (Physostigmine) and
ensure proper dosing/timing
 Monitor respir...
 During myasthenia crisis, the nurse should:
– Administer anti-cholinesterase medication as ordered.
– Assess and reasses...
Any Question?
THANK YOU
Myasthenia gravis
Myasthenia gravis
Myasthenia gravis
Myasthenia gravis
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Myasthenia gravis

Myasthenia Gravis By Hiren K Gehloth

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Myasthenia gravis

  1. 1. MYASTHENIA GRAVIS By: Hiren K Gehloth F.Y.M.Sc Nursing Govt.College Of Nursing,Abad(GINERA)
  2. 2. INTRODUCTION  Myasthenia gravis means “grave muscle weakness’ or “weakness of the voluntary or striated muscles of the body”.
  3. 3.  Myasthenia gravis (MG) is a relatively rare autoimmune disorder in which antibodies form against nicotinic acetylcholine (ACh) postsynaptic receptors at the neuromuscular junction (NMJ) of the skeletal muscles. It is a type-II hypersensitivity immune response.  The basic pathology is a reduction in the number of ACh receptors (AChRs) at the postsynaptic muscle membrane brought about by an acquired autoimmune reaction producing anti-AChR antibodies. The reduction in the number of AChRs results in a characteristic pattern of progressively reduced muscle strength with repeated use and recovery of muscle strength after a period of rest.
  4. 4.  The ocular and bulbar muscles are affected most commonly and most severely, but most patients also develop some degree of fluctuating generalized weakness.  The most important aspect of MG in emergency situations is acute worsening of weakness leading to neuromuscular respiratory failure. The diagnosis of myasthenic versus cholinergic crisis and its management is also a significant challenge in emergent settings.
  5. 5. DEFINITION  “Myasthenia Gravis is a auto immune disorder affecting the myoneural junction, characterized by varying degree of weakness of the voluntary muscle”
  6. 6. CAUSES AND RISK FACTORS  MG is idiopathic in most patients. Although the main cause behind its development remains speculative, the end result is a derangement of immune system regulation. MG is clearly an autoimmune disease in which the specific antibody has been characterized completely. In as many as 90% of generalized cases, IgG to AChR is present. Even in patients who do not develop clinical myasthenia, anti-AChR antibodies can sometimes be demonstrated.  Numerous findings have been associated with MG. For example, people with certain human leukocyte antigen (HLA) types have a genetic predisposition to autoimmune diseases.
  7. 7.  Thymic abnormalities are common: Of patients with MG, 75% have thymic disease, 85% have thymic hyperplasia, and 10–15% have thymoma. Extrathymic tumors may include small cell lung cancer and Hodgkin disease. Hyperthyroidism is present in 3–8% of patients with MG and has a particular association with ocular MG.
  8. 8. PATHOPHYSIOLOGY
  9. 9. TYPES OF MYASTHENIA GRAVIS → Congenital myasthenia gravis Congenital myasthenia gravis is defined by its cause: a genetic defect typically inherited from both parents, rather than an autoimmune disease in which immune system attacks damage communication between nerves and muscles — the cause of the other forms of this disease.
  10. 10. →Ocular myasthenia Gravis  In ocular myasthenia gravis, the muscles that move the eyes and control the eyelids are weakened and easily fatigued. Symptoms include drooping eyelids and double vision.
  11. 11.  While many patients with other forms of myasthenia gravis first experience symptoms in the muscles around the eyes as well, patients with ocular myasthenia gravis only have symptoms in these muscles, and the weakness and fatigue do not spread to other muscle groups. →Generalized myasthenia gravis  In patients with generalized myasthenia gravis, muscle weakness and fatigue are not confined to the eye muscles or other specific muscle groups. It may spread to the facial muscles as well as the limbs.
  12. 12.  In approximately 10 percent of these patients, muscle weakness spreads to the respiratory muscles or muscles of the throat and jaw. This can make breathing quite difficult, and can be extremely serious. Such episodes are called a “myasthenic crisis.” →Transient neonatal myasthenia gravis  Infants born to mothers with myasthenia gravis may develop symptoms of the disease about 48 hours after birth.  Symptoms can include impaired sucking and swallowing, a weak cry, and respiratory insufficiency. While serious, the symptoms typically disappear within days or weeks.
  13. 13. → Juvenile myasthenia gravis  In juvenile myasthenia gravis, symptoms begin before the onset of puberty.  Juvenile myasthenia gravis in its most benign form is confined to the eye muscles, but severe cases involving other muscle groups are known. Symptoms can range from clumsiness to difficulty in swallowing and tiring easily.  Young patients are less likely to test positive for the acetylcholine receptor antibodies that characterize generalized or ocular myasthenia gravis in adults. Juvenile myasthenia gravis patients are also more likely to achieve remission than adult patients, but periods of remission can give way to relapses.
  14. 14. CLINICAL MANIFESTAION  Drooping of one or both eye lids (ptosis)  Blurred or double vision  Breathing difficulty because of the chest wall muscle weakness.  Chewing swallowing difficulty, causing frequent gagging,chocking, drooling  Difficulty in climbing stairs, lifting objects, or rising from a seated position  Difficulty in talking(slurred speech)  Impaired speech (dysarthria)
  15. 15. •Drooping head •Facial paralysis or weakness of the facial muscle •Mask- like facial expression •Easy fatigability •Hoarseness or changing voice •Difficulty in maintain in steady gait •Weakness in arm, hands, fingers, legs, and neck.
  16. 16. DIAGNOSTICEVALUATION  A physical and neurological examination.  A blood test.  An Edrophonium test. This test uses injections of edrophonium chloride to briefly relieve weakness in people with myasthenia gravis. The drug blocks the breakdown of acetylcholine and temporarily increases the levels of acetylcholine at the neuromuscular junction. It is usually used to test ocular muscle weakness.
  17. 17. BEFORE AFTER
  18. 18.  Electrodiagnostics. Diagnostic tests include repetitive nerve stimulation, which repeatedly stimulates a person’s nerves with small pulses of electricity to tire specific muscles. Muscle fibers in myasthenia gravis, as well as other neuromuscular disorders, do not respond as well to repeated electrical stimulation compared to muscles from normal individuals. Single fiber electromyography (EMG), considered the most sensitive test for myasthenia gravis, detects impaired nerve-to-muscle transmission. EMG can be very helpful in diagnosing mild cases of myasthenia gravis when other tests fail to demonstrate abnormalities.  Diagnostic imaging. Diagnostic imaging of the chest using computed tomography (CT) or magnetic resonance imaging (MRI) may identify the presence of a thymoma.
  19. 19.  Pulmonary function testing. Measuring breathing strength can help predict if respiration may fail and lead to a myasthenic crisis.
  20. 20. MANAGEMENT Thymectomy- This operation to remove the thymus gland (which often is abnormal in individuals with myasthenia gravis) can reduce symptoms and may cure some people, possibly by rebalancing the immune system.
  21. 21.  A recent NINDS-funded study found that thymectomy is beneficial both for people with thymoma and those with no evidence of the tumors. The clinical trial followed 126 people with myasthenia gravis and no visible thymoma and found that the surgery reduced muscle weakness and the need for immunosuppressive drugs.  Anticholinesterase medications. Medications to treat the disorder include anticholinesterase agents such as mestinon or pyridostigmine, which slow the breakdown of acetylcholine at the neuromuscular junction and thereby improve neuromuscular transmission and increase muscle strength.
  22. 22.  Immunosuppressive drugs. These drugs improve muscle strength by suppressing the production of abnormal antibodies. They include prednisone, azathioprine, mycophenolate mofetil, tacrolimus, and rituximab. The drugs can cause significant side effects and must be carefully monitored by a physician.  Plasmapheresis and intravenous immunoglobulin. These therapies may be options in severe cases of myasthenia gravis. Individuals can have antibodies in their plasma (a liquid component in blood) that attack the neuromuscular junction. These treatments remove the destructive antibodies, although their effectiveness usually only lasts for a few weeks to months.
  23. 23.  Plasmapheresis is a procedure using a machine to remove harmful antibodies in plasma and replace them with good plasma or a plasma substitute.
  24. 24.  Intravenous immunoglobulin is a highly concentrated injection of antibodies pooled from many healthy donors that temporarily changes the way the immune system operates. It works by binding to the antibodies that cause myasthenia gravis and removing them from circulation.
  25. 25. COMPLICATION
  26. 26. NURSING MANAGEMENT  Administer cholinesterase inhibitors (Physostigmine) and ensure proper dosing/timing  Monitor respiratory status  Provide eye care  Monitor feeding and ensure proper nutrition. Schedule meds 30-45 minutes before meals.  Maintain suction and emergency equipment.  Educate patient to identify and avoid triggers  Educate patient on s/s of cholinergic and myasthenic crisis to report to provider.
  27. 27.  During myasthenia crisis, the nurse should: – Administer anti-cholinesterase medication as ordered. – Assess and reassess respiratory status. – Clear airway through thorough suctioning – Administration of supplemental oxygen as advised – Attach to ventilator assistance if necessary.  During cholinergic crisis, the nurse should: – Hold cholinergic drugs as it could exacerbate the situation. – Ensure airway clearance through prompt and thorough suctioning – Administer supplemental oxygen and ventilation assistance as needed – Strictly monitor hypotension
  28. 28. Any Question?
  29. 29. THANK YOU

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