Myasthenia gravis.PPTX of a BNS 3rd year

A presentation on a mysthania gravis.

1. Prepared by : Samikshya Banjara BNS 3 rd year 14 th batch Chitwan Medical College

2. Introduction The word “ Myasthenia Gravis” was derived by Latin and Greek: Myasthenia means muscle Gravis means weakness literally means “ Graves muscle weakness” 10/17/2024 BNS 3rd Year(14th batch) 2

3. Contd … Myasthenia gravis is a chronic autoimmune neuromuscular disease. It produces antibodies against Acetylcholine receptors( AchRs ) at Neuromuscular junction (NMJ). These antibodies attack and destroy AchRs and postsynaptic molecules Leads to impaired signal transduction Muscle weakness and fatigability 10/17/2024 BNS 3rd Year(14th batch) 3

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5. Definition Myasthenia gravis is a chronic autoimmune disorder characterized by fatigue and severe weakness of skeletal muscles that worsens with exercise and improves at rest. Myasthenia gravis , an autoimmune disorder affecting the myoneural junction, is characterized by varying degree of weakness of the voluntary muscles. 10/17/2024 BNS 3rd Year(14th batch) 5

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7. Contd … It usually affects proximal limb, bulbar and ocular muscles. The most affected muscles are eyes, face, and swallowing muscles. It can lead in double vision, drooping eyelids, trouble talking and trouble walking. MG is an acquired and probably heterogenous condition. The heart is not affected. 10/17/2024 BNS 3rd Year(14th batch) 7

8. Incidence MG occurs in either gender and in persons of any ethnicity. The worldwide prevalence of myasthenia gravis is 100-200 per million population, affecting more than 700,000 people all over the world. It may appear at any age. Although there are two peaks of onset. 10/17/2024 BNS 3rd Year(14th batch) 8

9. Contd … In early-onset MG, at age 20 to 30 years, women are more often affected than men. In late-onset MG, after age 50, men are more often affected. (AANN, 2013; Bader & Little Johns,2010). 10/17/2024 BNS 3rd Year(14th batch) 9

10. Causes Exact cause is unknown In about 90% of patients, no specific cause can be identified, but genetic make-up is a predisposing factor . Environmental factors may be involved in development of this disorder. 10/17/2024 BNS 3rd Year(14th batch) 10

11. Contd … Autoantibodies blocks the receptors of acetylcholine in neuromuscular junction. 10/17/2024 BNS 3rd Year(14th batch) 11

12. Contd … Brittle factors occurs when the receptors at the neuromuscular junction become insensitive to Anticholinestrase medication. 10/17/2024 BNS 3rd Year(14th batch) 12

13. Contd … Viral Infection Aging Factors Women are three times more susceptible to developing myasthenia gravis. Pregnancy Menstruation 10/17/2024 BNS 3rd Year(14th batch) 13

14. Contd … Thyroid gland abnormalities are present in 80% of patients. Thymic tumor is the most important known cause; 20% of patients with myasthenia gravis have a thymoma. Thymoma : enlargement of thymus 10/17/2024 BNS 3rd Year(14th batch) 14

15. Contd … Autoantibodies against musk protein tyrosine kinase receptor which helps in neuromuscular junction formation. 10/17/2024 BNS 3rd Year(14th batch) 15

16. Pathophysiology Normal Neuro Muscular transmission Motor nerve impulses travel to motor nerve terminal Acetylcholine(ACh) is released ACh diffuses across synapse ACh receptor sites in motor end plates depolarize muscle fiber Depolarization spreads, causing muscle contraction 10/17/2024 BNS 3rd Year(14th batch) 16

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18. Neuro muscular transmission in MG 10/17/2024 BNS 3rd Year(14th batch) 18

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20. Signs and Symptoms Fatigue, muscle weakness involving specific group of muscles is the clinical hallmark of myasthenia gravis. 10/17/2024 BNS 3rd Year(14th batch) 20

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22. Signs and Symptoms contd .. Ocular muscles Fluctuating ptosis Diplopia Bulbar muscles Dysarthia , Painless Dysphagia , Dysphonia Masticulatory weakness, Choking 10/17/2024 BNS 3rd Year(14th batch) 22

23. Signs and Symptoms contd.. Facial muscle Facial weakness Inability to close eyes firmly Drooling of saliva Axial muscle Difficulty in flexion and extension of neck 10/17/2024 BNS 3rd Year(14th batch) 23

24. Signs and Symptoms contd.. Limb muscles Weakness involving arms more than legs Respiratory muscles Labor breathing Dyspnea And respiratory failure. 10/17/2024 BNS 3rd Year(14th batch) 24

25. Signs and Symptoms contd .. This weakness usually fluctuate from hour to hour, day to day,( snowflake disease ) worsen with activity and improves on rest. 10/17/2024 BNS 3rd Year(14th batch) 25

26. Classifications of MG Myasthenia gravis is classified clinically into five classes and several subclasses according to the “ Myasthenia Gravis foundation of American clinical classification ”: 10/17/2024 BNS 3rd Year(14th batch) 26

27. Class Clinical description Class 1 Any eye muscle weakness, possible ptosis and all other muscle strength is normal. Class 2 Mild weakness of other muscles, may have eye muscle weakness of any severity Class2a Predominantly limb and axial muscle weakness or both Class2b Predominantly Oropharyngeal or respiratory muscle weakness or both 10/17/2024 BNS 3rd Year(14th batch) 27

28. Class Clinical Description Class3 Eye muscle weakness of any severity, moderate weakness of other muscles. Class3a Predominantly limb and axial muscle weakness Class3b Predominantly bulbar or respiratory muscle weakness Class4 Eye muscle weakness of any severity, severe weakness of other muscles. 10/17/2024 BNS 3rd Year(14th batch) 28

29. Class Clinical Description Class4a Predominantly limb and axial muscle weakness Class4b Predominantly bulbar or respiratory muscle weakness Class5 Intubation needed to maintain airway 10/17/2024 BNS 3rd Year(14th batch) 29

30. Diagnostic Investigations History Taking : History include complains of fatigue, weakness, medications intake, menstrual periods, pregnancy, viral infection . Physical Examination : Cranial Nerve function, motor fatigability, Speech, Vital signs(esp. Respiratory rate) 10/17/2024 BNS 3rd Year(14th batch) 30

31. Diagnostic Investigations contd.. Acetylcholine receptor antibody test :Abnormally elevated, Serum test for acetylcholine receptor ( AChR ) antibodies positive in up to 90% of patients. Computed tomography or MRI : It may be used to identify the normal thymus gland or the presence of thymoma. 10/17/2024 BNS 3rd Year(14th batch) 31

32. Contd.. Edrophonium ( Tensilon ) test : This approach requires the intravenous administration of edrophonium chloride, a drug that blocks the degradation of acetylcholine receptors and temporarily increase the level of acetylcholine in neuromuscular junction. This test relieves symptoms temporarily. After injection, a marked but temporary improvement in muscle strength suggests myasthenia gravis. 10/17/2024 BNS 3rd Year(14th batch) 32

33. Contd … A test dose of 2mg (for adult)is injected first If no untoward reaction occurs (increase weakness, change in heart rate, nausea or abdomen pain), the remaining 8mg is injected Observe for improvement in muscle strength 10/17/2024 BNS 3rd Year(14th batch) 33

34. Diagnostic Investigations contd.. Pulmonary function test : It measures breathing strength, helps to predict whether respiration may fail and leads to myasthenia gravis. 10/17/2024 BNS 3rd Year(14th batch) 34

35. Electromyography : Muscles fiber in myasthenia gravis and other neuromuscular disorder, do not respond as well to repeated electrical stimulation compared to muscles of normal person. 10/17/2024 BNS 3rd Year(14th batch) 35

36. Contd.. Ice Pack Test : Cooling may improve Neuromuscular transmission in a patient with myasthenia gravis who has ptosis, placing ice over an eyelid will lead to cooling of the lid, which leads to improvement of the ptosis 10/17/2024 BNS 3rd Year(14th batch) 36

37. Diagnostic Investigations contd.. Blood analysis : Elevated levels of acetylcholine receptor antibodies 10/17/2024 BNS 3rd Year(14th batch) 37

38. Management: No cure for Myasthenia Gravis as yet. Objectives or Goals are to achieve the maximum benefit with fewest side effects and to improve muscles strength Medical management(Pharmacological) Surgical management Nursing management 10/17/2024 BNS 3rd Year(14th batch) 38

39. Medical Management Anticholinesterase drugs : Anticholinesterase drugs inhibit cholinesterase, the enzyme responsible for the breakdown of acetylcholine allowing enhanced receptor stimulation . As a result, more acetylcholine is available to effect neuromuscular transmission. Drugs Include: 10/17/2024 BNS 3rd Year(14th batch) 39

40. Medical Management Neostigmine ; which helps to improve neuromuscular transmission and increase muscle strength. Pyridostigmine ( mestinon ) is the most successful drug of this group in long-term treatment of MG. Steroids: this is given to decrease inflammation. 10/17/2024 BNS 3rd Year(14th batch) 40

41. Medical Management contd.. Immunosuppressive drugs; drugs such as prednisolone and cyclosporine are used. It improves muscle strength by suppressing the production of abnormal antibodies Immunoglobulins : this is given through iv infusion to boost up immune system. 10/17/2024 BNS 3rd Year(14th batch) 41

42. Contd.. Plasmapheresis It is the procedure in which the abnormal antibodies are removed from the blood. 10/17/2024 BNS 3rd Year(14th batch) 42

43. Surgical Management: Thymectomy: Surgical removal of thymus gland can be done. 10/17/2024 BNS 3rd Year(14th batch) 43

44. Nursing Management

45. Assessment History Taking : History include complains of fatigue, weakness, medications intake, menstrual periods, pregnancy, viral infection . Physical Examination : Cranial Nerve function, motor fatigability, Speech, Vital signs(Resp) 10/17/2024 BNS 3rd Year(14th batch) 45

46. Assessment contd.. Assess the muscle weakness and fatigue. Assess cranial nerve function, motor fatigability with repetitive activity, and speech. Observe eye muscles (usually affected first) for ptosis, ocular palsy, diplopia. Assess respiratory status breathlessness, respiratory weakness, tidal volume, and vital capacity measurements. 10/17/2024 BNS 3rd Year(14th batch) 46

47. Nursing diagnosis

48. Actual diagnosis 10/17/2024 BNS 3rd Year(14th batch) 48

49. Actual diagnosis 10/17/2024 BNS 3rd Year(14th batch) 49

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52. Nursing Diagnosis 10/17/2024 BNS 3rd Year(14th batch) 52

53. Nursing Intervention

54. To maintain breathing pattern Place patient with proper body alignment for maximum breathing pattern. Encourage sustained deep breaths. Techniques include: highlighting slow inhalation, holding end inspiration for a few seconds, and passive exhalation; (2) utilizing incentive spirometer and (3) requiring the patient to yawn. 10/17/2024 BNS 3rd Year(14th batch) 54

55. Contd … Encourage diaphragmatic breathing for patients Avoid high concentrations of oxygen in patients with COPD. Maintain a clear airway. Suction secretions, as necessary. 10/17/2024 BNS 3rd Year(14th batch) 55

56. 2. To improve physical mobility Assist patient for muscle exercises as able or when allowed out of bed; execute abdominal-tightening exercises and knee bends; stand on toes. Provide a safe environment: bed rails up, bed in a down position, important items close by. 10/17/2024 BNS 3rd Year(14th batch) 56

57. Contd.. Establish measures to prevent skin breakdown and thrombophlebitis from prolonged immobility Execute passive or active assistive ROM exercises to all extremities 10/17/2024 BNS 3rd Year(14th batch) 57

58. Contd.. Show the use of mobility devices, such as the following: trapeze, crutches, or walkers. Give positive reinforcement during activity. Patients may be unwilling to move or initiate new activity because of fear of falling. 10/17/2024 BNS 3rd Year(14th batch) 58

59. 3. For selfcare deficit Assess the patient’s strength to accomplish ADLs efficiently Guide the patient in accepting the needed amount of dependence. Provide positive reinforcement for all activities attempted; note partial achievements. 10/17/2024 BNS 3rd Year(14th batch) 59

60. Contd.. Implement measures to promote independence, donot but intervene when the patient cannot function. Apply regular routines, and allow adequate time for the patient to complete task. 10/17/2024 BNS 3rd Year(14th batch) 60

61. 4. Maintaining social interactions Encourage patient to use an alternate communication method, such as flash cards or a letter board, if speech is affected. Instruct patient to speak in a slow manner to avoid voice strain; refer to speech therapy as needed. 10/17/2024 BNS 3rd Year(14th batch) 61

62. Contd.. Teach patient to tilt head, and to carry a handkerchief to manage secretions in public. Encourage family participation in care. Refer patient to the Myasthenia Gravis Foundation to meet other patients with the disease who lead productive lives. 10/17/2024 BNS 3rd Year(14th batch) 62

63. 5. To minimize Fatigue Plan exercise, meals, and other ADLs during energy peaks. Administration of medications 30 minutes before meals to facilitate chewing and swallowing. Assist the patient in developing realistic activity schedule 10/17/2024 BNS 3rd Year(14th batch) 63

64. Contd … Provide an eye patch, and alternate eyes for the patient with diplopia to allow safe participation in activity. Allow for rest periods throughout the day. Obtain assistive devices to help patient perform ADLs. 10/17/2024 BNS 3rd Year(14th batch) 64

65. 6. To prevent Aspiration Assess patient's oral motor strength before each meal. Teach patient to position head in a slightly flexed position to protect airway during eating. Modify diet as needed to minimize risk of aspiration; for instance, soft, solid foods instead of liquid. Teach patient that eating warm foods (not hot foods) can ease swallowing difficulties. Have suction available at bed side. 10/17/2024 BNS 3rd Year(14th batch) 65

66. Contd.. Suction the patient frequently if on a mechanical ventilator Administer I.V. fluids and nasogastric tube feedings to patient in crisis or with impaired swallowing; elevate head of bed after feeding. Suction the patient frequently if on a mechanical ventilator; assess breath sounds and check chest X-ray reports because aspiration is a common problem Never speak while eating 10/17/2024 BNS 3rd Year(14th batch) 66

67. Contd … Deep breathing and coughing exercise 10/17/2024 BNS 3rd Year(14th batch) 67

68. Teach patient and family how to use home suction in case of aspiration. Make sure everyone in household knows Heimlich maneuver.(Discovered by American doctor, Henry Heimlich) It is a first aid to treat upper airway obstruction. Others 10/17/2024 BNS 3rd Year(14th batch) 68

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70. Community and Home Care Considerations Assess home environment for physical and emotional stressors, such as uncomfortable temperature, draft, loud noises, and encourage avoidance of such. Emphasize continued follow-up and compliance with treatment regimen. 10/17/2024 BNS 3rd Year(14th batch) 70

71. Contd … Make referrals to community agencies as indicated, such as home respiratory care, physical therapy, nutritional services. Assess patient frequently for fluctuation in condition , and inform caregivers that this is common. 10/17/2024 BNS 3rd Year(14th batch) 71

72. Contd … Teach patient and family how to use home suction in case of aspiration. Make sure everyone in household knows Heimlich maneuver. 10/17/2024 BNS 3rd Year(14th batch) 72

73. Complications: Myasthenia crisis Cholinergic crisis 10/17/2024 BNS 3rd Year(14th batch) 73

74. Myasthenia crisis Myasthenic crisis is a life-threatening condition that is defined as worsening of myasthenic weakness requiring intubation or noninvasive ventilation. 10/17/2024 BNS 3rd Year(14th batch) 74

75. Myasthenia crisis contd.. While the respiratory failure is due to weakness of respiratory muscles, oropharyngeal muscle weakness often accompanies the respiratory muscle weakness, or may be the predominant feature in some patients. When this results in upper airway obstruction or severe dysphagia with aspiration, intubation and mechanical ventilation are necessary. 10/17/2024 BNS 3rd Year(14th batch) 75

76. Cholinergic crisis This is usually secondary to the inactivation or inhibition of Acetylcholinesterase ( AchE ), the enzyme responsible for the degradation of acetylcholine (ACh). Excessive accumulation of acetylcholine (ACh) at the neuromuscular junctions and synapses causes symptoms of both muscarinic and nicotinic toxicity. 76 10/17/2024 BNS 3rd Year(14th batch)

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79. Prognosis: If the patient is treated properly in early stage, the prognosis is good. However continuous observation should be done as the person may experience myasthenia crisis in later stage. Most of the people with myasthenia gravis died due to different respiration related problems. 10/17/2024 BNS 3rd Year(14th batch) 79

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81. Reference Basavanthappa, BT. Medical Surgical Nursing (2nded.) Newdelhi : Jaypee Brothers Medical Publishers (p) Ltd. 822-824. Black, M.J & Hawks, H.J (2015). Medical –Surgical Nursing.(8thed.) volume-2 , India, Haryana: Reed Elsevier India Private Limited.1842-1843 10/17/2024 BNS 3rd Year(14th batch) 81

82. Contd … Sharma, S.K, S. Hinkle, J.K., Cheever, K.H (2018),Brunner & suddarth’s Textbook of Medical Surgical Nursing Volume 2 (13th edition ), South Asian edition. Wolters Kluwer Health (1964-1965) Sharma, T.S & Madhavi, S (2018). Brunner & suddarth’s Textbook of Medical – Surgical Nursing. South Asia(ed.). Newdelhi , India: Wolters Kluwer Pvt. Ltd. 1786-1770. 10/17/2024 BNS 3rd Year(14th batch) 82

83. Contd … Shrestha, H., Paudyal , P. & Giri , S. (2072). A textbook of Medical Surgical Nursing I & II. 2nd (ed.), Kathmandu, Nepal: Heritage Publishers & distributors Pvt. Ltd 378- 379. Smeltzer, C.S., Bare, G.B, Hinkle, L.J., & Cheever, H.K (2011). Brunner & suddarth’s Textbook of Medical – Surgical Nursing. 12th (ed.). Wolters Kluwer Pvt. Ltd 1964- 1965. 10/17/2024 BNS 3rd Year(14th batch) 83

84. Contd … Wlindsay, k &Bone, L. Neurology and Neurosurgery illustrated (5th edition). Churchill Living stone Elsevier (484-487) Net References https://www.slideshare.net/mdyaqub16/myasthenia-gravis-71122636 10/17/2024 BNS 3rd Year(14th batch) 84

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