myasthenia gravis.pptx

myasthenia gravis , a neurological disorder, causes skeletal muscle weakness. There are classification according to american clinical classification of myasthenia gravis.Risk factors and causes of myasthenia gravis with animated gif shown in ppt. Types of muscle weakness and pathophysiology of myasthenia gravis explained. Clinical manifestation explained through animated gif. Important diagnostic test explained through pictures. Medical management, surgical management, nursing management explain in detail of myasthenia gravis. Excercise goals and rehablitation management of myasthenia gravis is explained. Types of rehablitation excercise for myasthenia gravis explained. Complications of myasthenia gravis and research article of myasthenia gravis is included in ppt. Summary and conclusion is also included in ppt.

1. KING GEORGE’S MEDICAL UNIVERSITY K.G.M.U. COLLEGE OF NURSING MYASTHENIA GRAVIS PRESENTED BY- DIVYA PAL M.Sc. NURSING I YEAR

2. OBJECTIVES Introduction Definition Incidence Risk factors Causes Types Pathophysiology Sign and symptoms Diagnostic Evaluation Medical Management Surgical Management Nursing Management Complication

3. INTRODUCTION Myasthenia Gravis is an autoimmune disease of the neuromuscular junction c haracterized by the fluctuating weakness of the certain skeletal muscle, group. It results from a loss of acetylcholine receptors in the postsynaptic neurons of the neuromuscular junction.

4. DEFINITION Myasthenia gravis is a chronic autoimmune, neuromuscular disease that causes weakness in the skeletal muscles (the muscles that connect to your bones and contract to allow body movement in the arms and legs, and allow for breathing).

5. INCIDENCE Myasthenia Gravis affects 1 or 2 people in every 10,000. It occurs more commonly in women under 40 years and men over 60. However, it may affect individuals of any age and we have largest experience in India for treatment of Myasthenia Gravis.

6. CLASSIFICATION Myasthenia Gravis Foundation of America Clinical Classification[10] 1 . Class I: Any ocular muscle weakness, possible ptosis, no other evidence of muscle weakness elsewhere. 2. Class II: Mild weakness affecting other than ocular muscles; may also have ocular muscle weakness of any severity. 3. Class Ila: Predominantly affecting limb, axial muscles, or both; may also have lesser involvement of oropharyngeal muscles

7. CLASSIFICATION 7. Class Ilb : Predominantly bulbar and/or respiratory mack to to top also have lesser or equal involvement of limb, axial muscle or both. 8. Class IV: Severe weakness affecting other than ocular muscles; may also have ocular muscle weakness of any severity. 9. Class IVa : Predominantly affecting limb, axial muscles, or both; may also have lesser involvement of oropharyngeal muscles.

8. CLASSIFICATION 10 . Class IVb : Predominantly bulbar and/or respiratory muscles; may also have lesser or equal involvement of limb, axial muscles, or both (Can also include feeding tube without intubation). 11. Class V: Intubation needed to maintain airway, with or without mechanical ventilation.

9. RISK FACTORS Fatigue S tress Surgery Illness or infection

10. RISK FACTORS Some medications such as beta blockers, quinidine gluconate, quinidine sulfate , quinine ( Qualaquin ), phenytoin, certain anesthetics and some antibiotics Pregnancy Menstrual periods

11. CAUSES Antibodies that destroys acetylcholine receptors. Genetic Unknown

12. TYPES Facial muscle weakness Bulbar muscle weakness. Limb muscle weakness Respiratory weakness Occular muscle weakness Extraoccular muscle weakness

13. FACIAL MUSCLE WEAKNESS It includes head extension and flexion weakness. Weakness may worse in proximal muscle.

14. EXTRAOCCULAR MUSCLE WEAKNESS Ptosis is present initially in 50% of the patients and during course of disease in 90% of patients.

15. BULBAR MUSCLE WEAKNESS Group of muscles in head and neck. Palatal muscles Nasal voice ,nasal Regurgitation. Chewing become difficult. Severe jaw weakness may cause jaw to hang open.

16. LIMB MUSCLE WEAKNESS Upper limbs more Common than Lower limbs.

17. RESPIRATORY MUSCLE WEAKNESS Weakness of the intercostal space muscles and the diaphragm may result in Co2 retention due to hypoventilation. Weakness of pharyngeal muscles may collapse the upper airway.

18. OCCULAR MUSCLE WEAKNESS Assymetric :- Usually affects more than one extraoccular muscles and is not limited to muscles innervated by one cranial nerve. Weakness of lateral and medied recti may produce & a pseudo internuclear opthalmoplegia . Ptosis caused by eyelid weakness. Diplopia is very common.

19. PATHOPHYSIOLOGY Due to etiological Factor Lymphocyte produce Ach Receptor antibodies that attack the postsynaptic muscle membrane. Depletion of acetylcholine receptors of the neuromuscular junction

20. PATHOPHYSIOLOGY Defect in transmission of impulse from nerve to muscle cell Myasthenia Gravis

21. CLINICAL MANIFESTATION Increasing muscle weakness affects movements of eyes, chewing, swallowing, speaking and breathing Ptosis (drooping of one or both eyelids)

22. CLINICAL MANIFESTATION Laryngeal involvement produces dysphonia. Changes in facial expression Diplopia

23. CLINICAL MANIFESTATION Shortness of breath Impaired speech Weakness in arms, hands, fingers, legs and neck

24. DIAGNOSTIC EVALUATION History collection – A ge and gender- The early type (before age of 50) is female predominant and the late type (after age of 60) is male dominant. Race- Some studies demonstrated that the incidence, prevalence and the severity of this disease is higher inAfrican /Americans. Evidence of coexisting autoimmune diseases.

25. DIAGNOSTIC EVALUATION A positive history of- Heavy eyelids and double vision Eye movement problems Photophobia Facial weakness

26. DIAGNOSTIC EVALUATION Tongue weakness, chewing and swallowing problems Respiratory problems Limbs muscles weakness Fatigue Urinary incontinency

27. DIAGNOSTIC EVALUATION Physical examination- Muscle strength and tone Co-ordination Sense of touch Any impairment of eye movement

28. DIAGNOSTIC EVALUATION Edrophonium Tensilon test- This test is used to test eye muscle weakness and uses injections of edrophonium chloride to briefly relieve weakness. The drug blocks the breakdown of acetylcholine and temporarily increases the levels of acetylcholine at the neuromuscular junction.

29. DIAGNOSTIC EVALUATION Blood test- People living with myasthenia gravis may have abnormally elevated levels of acetylcholine receptor antibodies. A second antibody called the anti- MUSK antibody has been found in about half of individuals with myasthenia gravis who do not have acetylcholine receptor antibodies. A blood test can also detect this antibody.

30. DIAGNOSTIC EVALUATION Electrodiagnostics - Diagnostic tests include repetitive nerve stimulation, which repeatedly stimulates 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 .

31. DIAGNOSTIC EVALUATION Single fiber Electromyography- It is most sensitive test for Myasthenia Gravis. It detects impaired nerve- to- muscle transmission.

32. DIAGNOSTIC EVALUATION Diagnostic imaging- Diagnostic imaging of the chest using computed tomography (CT) or magnetic resonance imaging (MRI) may identify the presence of a thymoma.

33. DIAGNOSTIC EVALUATION CT Scan

34. DIAGNOSTIC EVALUATION M.R.I

35. DIAGNOSTIC EVALUATION Pulmonary function test - Measuring breathing strength can help predict if respiration may fail and lead to a myasthenic crisis.

36. DIAGNOSTIC EVALUATION Ice pack test- When edrophonium test is contraindicated , used for patients with ptosis.

37. MEDICAL MANAGEMENT Cholinesterase inhibitor Corticosteroid Immunosuppresants Plasmapheresis IVIg Monoclonal Antibodies

38. MEDICAL MANAGEMENT Cholinesterase Inhibitor- Mestinon or pyridostigmine . It slows down breakdown of Acetylcholine at the neuromuscular junction and improve neuromuscular transmission and increase muscle strength.

39. MEDICAL MANAGEMENT Immunosuppressive drugs-Group of drugs that improve muscle strength by suppressing the production of abnormal antibodies, such as prednisone, azathioprine, mycophenolate mofetil, and tacrolimus.

40. MEDICAL MANAGEMENT Plasmapheresis and intravenous immunoglobulin-Therapies that are used in severe cases of myasthenia gravis to remove destructive antibodies that attack the neuromuscular junction, although their effectiveness usually only lasts a few weeks or months.

41. MEDICAL MANAGEMENT https://youtu.be/oTKOxfhJ6AQ

42. MEDICAL MANAGEMENT Monoclonal Antibodies -A treatment that targets the process by which acetylcholine antibodies injure the neuromuscular junction. The U.S. Food and Drug Administration (FDA) has approved the use of the medication eculizumab for the treatment of generalized myasthenia gravis in adults who test positive for the antiacetylcholine receptor ( AchR ) antibody.

43. SURGICAL MANAGEMENT Thymectomy -An operation to remove the problematic thymus gland can reduce symptoms, possibly by rebalancing the immune system. Myasthenia gravis with thymoma is treated.

44. MANAGEMENT STRATEGIES FOR REHABLITATION Treatment strategies may vary. Evaluation is done on the basis of- Strength Flexibility Mobility Balance Gait

45. Benefits of Exercise in Myasthenia Gravis Weight reduction. Decrease in risk of hypertension, diabetes, cholesterol Decrease in risk of cardiac diseases

46. Decrease in risk of osteoporosis Mood elevation I mprove cognitive function Enhance baseline functional capacity, improved mechanical efficiency

47. EXERCISE GOALS Enhance ability to function daily Decrease risk of falling Completion of functional tasks and maintenance of independence Smoothness and coordination of activities

48. EXERCISE CONSIDERATION Exercise at the best time of day Exercise at peak dose of medication (pyridostigmine) Exercise large, proximal muscle groups for short periods of time building up only to moderate intensity Do not exceed moderate intensity exercise level

49. Nurses Responsibility During Exercise- Patient should not become short of breath at peak of exercising Myasthenia Gravis symptoms should not become worse during exercise (drooping of eyes) Patient should not be tires after 2 hours of exercise

50. REHABLITATION EXERCISE PACE is a suitable guideline. P- Planning daily activities A-Adapting the home ,adequate rest ,assistive equipment, asking for help. C- Conserving energy, Check ups with doctor, cool temperature E- Emotional stability, Excercising in moderation, eliminating unnecessary daily tasks.

51. TYPES OF EXCERCISE Types of Exercise used in Myasthenia Gravis- Breathing Exercises Postural Exercises Aerobic Excercises Strength Excercises Swimming

52. TYPES OF EXCERCISE Breathing excercises - To strengthen the muscles that control the voice. Vocal excercises to improve the ability to speak clearly. Help to improve lung function. It include respiratory muscle training- Pursed lip breathing Diaphragmatic breathing

53. TYPES OF EXCERCISE Postural Exercises- Important in assisting with breathing, speaking and swallowing Keeps bones and joints in the correct alignment so that muscles are being used properly Prevents fatigue because muscles are being used more efficiently, allowing the body to use less energy

54. TYPES OF EXCERCISE Strengthen Exercise - Should be done progressively Range of motion (flexibility) to light resistance to full resistance If significant weakness is present, active assist exercises may be necessary. The primary goal of therapy is to build the individual's strength to facilitate return to work and activities of daily living.

55. TYPES OF EXCERCISE Swimming - Patients should swim in water where they can touch the bottom. Deep water is dangerous and may cause patient to over exert.

56. NURSING MANAGEMENT Monitor respiratory status and ability to cough and deep breathe adequately. Monitor for respiratory failure. Maintain suctioning and emergency equipment at the bedside. Monitor vital signs. Monitor speech and swallowing abilities to prevent aspiration.

57. NURSING MANAGEMENT Encourage the client to sit up when eating. Assess muscle status. Instruct the client to conserve strength. Plan short activities that coincide with times of maximal muscle strength. Monitor for myasthenic and cholinergic crises.

58. NURSING MANAGEMENT Administer anticholinesterase medications as prescribed. Instruct the client to avoid stress, infection, fatigue, and over-the counter medications. Instruct the client to wear a Medic-Alert bracelet. Inform the client about services from the Myasthenia Gravis Foundation .

59. COMPLICATIONS Myasthenic Crisis Thyroid gland tumor

60. NURSING DIAGNOSIS Ineffective airway clearance related to decreased ability to cough or swallow as evidenced by absent or ineffective cough. Impaired breathing pattern related to disease process as evidenced by Presence of adventitious sounds. Fatigue related to muscle weakness as evidenced by lethargy. Activity intolerance related to low self esteem as evidenced by weakness. Risk for aspiration related to depressed gag reflex .

61. RESEARCH ARTICLE “Global prevalence of myasthenia gravis and the effectiveness of common drugs in its treatment: a systematic review and meta- analysis.” Salari N et al. Transl Med. 2021. PMID:34930325

62. RESEARCH ARTICLE Background: Myasthenia gravis is a neuromuscular autoimmune disorder characterized by weakness and disability in the voluntary muscles. There have been several preliminary studies on the epidemiology of myasthenia gravis in different parts of the world and the effectiveness of common drugs in its treatment using systematic review and meta- analysis.

63. RESEARCH ARTICLE Methods- Research studies were extracted from IranDoc , MagIran , Iran Medex , SID, ScienceDirect, Web of Sciences ( WoS ), ProQuest, Medline (PubMed), Scopus and Google Scholar based on Cochran's seven- step guidelines using existing keywords extracted in MeSH browser. The 12 test was used to calculate the heterogeneity of studies, and Begg and Mazumdar rank correlation tests were used to assess publication bias. Data were analyzed using Comprehensive Meta-Analysis software (Version 2).

64. RESEARCH ARTICLE Results- In the search for descriptive studies based on the research question, 7374 articles were found. After deleting articles unrelated to the research question, finally, 63 articles with a sample size of 1,206,961,907 people were included in the meta-analysis. The prevalence of MG worldwide was estimated to be 12.4 people (95% CI 10.6-14.5) per 100,000 population.

65. RESEARCH ARTICLE . For analytical studies on the effectiveness of common myasthenia gravis drugs, 4672 articles were found initially, and after removing articles unrelated to the research question, finally, 20 articles with a sample size of 643 people in the drug group and 619 people in the placebo group were included in the study. As a result of the combination of studies, the difference between the mean QMGS score index after taking Mycophenolate and Immunoglobulin or plasma exchange drugs in the group of patients showed a significant decrease of 1.4 ± 0.77 and 0.62 ± 0.28, respectively (P < 0.01). and Immunoglobulin or plasma exchange drugs in the group of patients showed a significant decrease of 1.4 ± 0.77 and 0.62 ± 0.28, respectively (P < 0.01).

66. RESEARCH ARTICLE Conclusion- The results of systematic review of drug evaluation in patients with myasthenia gravis showed that Mycophenolate and Immunoglobulin or plasma exchange drugs have positive effects in the treatment of MG. It also represents the positive effect of immunoglobulin or plasma exchange on reducing SFEMG index and QMGS index and the positive effect of Mycophenolate in reducing MG-ADL index, SFEMG and Anti- AChR antibodies index. In addition, based on a meta-analysis of the random-effect model, the overall prevalence of MG in the world is 12.4 people per 100,000 population, which indicates the urgent need for attention to this disease for prevention and treatment .

67. SUMMARY Myasthenia gravis is an autoimmune disease affecting the neuromuscular junction. It causes muscle weakness. Myasthenia gravis may affect individuals of any age and we have largest experience in India for treatment of Myasthenia gravis.

68. CONCLUSION As discussed throughout the presentation, learning about myasthenia gravis and its management will help nurses to care for a myasthenia gravis patient. Nurses can do assessment of a myasthenia gravis patient, classify the level of disease, observe the sign and symptoms, provide the necessary nursing care and support the patient psychologically.

69. RECAPITULATION What is Myasthenia Gravis? What are the causes and sign and symptoms of Myasthenia Gravis? Write the short note on communication and rehabilitation management of Myasthenia Gravis.