Myasthenia gravis during pregnancy

2. Items to be discussed in this talk • What is Myasthenia Gravis? • Incidence • Etiology • Clinical presentation • Examination • Investigations • Clinical classification • Life threatening presentations • Management • Myasthenia Gravis AND pregnancy • Trivia

4. What is Myasthenia Gravis? • MYO = MUSCLE • ASTHENIA = WEAKNESS • GRAVIS = SEVERE Myasthenia Gravis = Severe Muscle Weakness

5. What is Myasthenia Gravis? • MG is an autoimmune neuromuscular disease leading to fluctuating muscle weakness and fatigue.

7. Incidence • 1 in 7’500 • Mainly affects females • Age: 20 – 30 years

9. Etiology • Autoimmune: IgG vs Acetyl choline receptors (AChR) diminished end-plate action potential  weak muscle contractions

11. Clinical presentation • The characteristic feature is Fatigability • Initially intermittent symptoms: EYE • Within 1 year: Generalized (85%) • Remissions and Exacerbations

12. Clinical presentation • Eye: Diplopia - Ptosis (Early, Common, Symmetrical or Assymetrical) • Bulbar: Dysphagia – Dysarthria • Face: Difficult smiling & chewing • Respiratory: Dyspnea • Limb: Weakness – Gait • Heart: Not affected

14. Examination • Muscle strength: Weakness • Deep tendon reflexes: Normal • Associated autoimmune diseases: SLE, RA, DM, Thyroid

16. Investigations • Edrophonium test: THERAPEUTIC TEST Cholinesterase inhibitor  ↑ available ACh  Clinical improvement occurs in 10-60 sec and lasts for approximately 10 min • Anti-AChR antibody: MOST SPECIFIC Sensitivity: 80-95% except ocular MG (50%) • EMG • Pulmonary function tests • CT or MRI: Thymoma (75%) • Screen for other autoimmune diseases

18. Classification • Class I: Eye muscle weakness only • Class II: Eye muscle weakness + mild weakness of other muscles • Class III: Eye muscle weakness + moderate weakness of other muscles • Class IV: Eye muscle weakness + severe weakness of other muscles OR need for nasogastric feeding • Class V: Intubation needed to maintain airway

20. Life threatening presentations Crises may be precipitated by: • Systemic diseases • Concurrent infections • Emotional upset

21. Life threatening presentations • Myasthenic crises: severe muscle weakness, inability to swallow, and respiratory muscle paralysis • Refractory crises: the same symptoms but unresponsive to the usual therapy • Cholinergic crises: excessive cholinergic medication leads to nausea, vomiting, muscle weakness, abdominal pain, and diarrhea.

23. Management • Medications • Plasmapharesis • Surgery

25. Medications • Anti-choline esterase: - Neostigmine and Pyridostigmine - Action: slowing cholinesterase that degrades acetylcholine in the motor end plate  becomes present longer to stimulate its receptor  improve muscle function - Dose & Administration: Start with a low dose, e.g. 3 x 20 mg pyridostigmine, and increase until the desired result is achieved. If taken 30 minutes before a meal, symptoms will be mild during eating. - Side effects: Perspiration and diarrhea - Toxicity: Cholinergic crises (D.D. Myasthenic crisis)

26. Medications • Immunosuppressives: - Corticosteroids e.g. Prednisone - Azathioprine - Cyclosporin - Mycophenolate - Cyclophosphamide for refractory cases • Immunoglobulin therapy: - 0.4 gm/kg/day for 5 days • Pulse steroid: - Methylprednisolone Myasthenic crisis OR Before surgery

28. Plasmapharesis - Effective - Expensive For: - Myasthenic crisis - Before surgery

30. Surgery Thymectomy For: Thymoma OR Thymic hyperplasia (75%)

32. Myasthenia Gravis AND Pregnancy • Effect of pregnancy on MG. • Effect of MG on pregnancy & labor. • Pre-conceptional management. • Antenatal management. • Management during delivery. • Postpartum management

34. Effect of pregnancy on MG • Unpredictable: Worsens, improves or unchanged. • Risks: Exacerbation, Crises, Respiratory complications necessitating ventilation, Death • Most dangerous in 1st ∆

36. Effect of MG on pregnancy • Maternal: - Pre-eclampsia (Immunological theory) ? - Hydramnios ? - Prolonged 2nd stage (Can’t bear down) - ↑ C.S. • Fetal: - IUGR - PTL - Miscarriage & Still birth - Neonatal MG (10-20%)

37. Myasthenia Gravis AND Pregnancy • Effect of pregnancy on MG. • Effect of MG on pregnancy. • Pre-conceptional management. • Antenatal management. • Management during delivery. • Postpartum management

38. Pre-conceptional management • Counselling about risks (maternal-fetal) • Pregnancy better avoided during 1st year • Pregnancy better postponed till a period of remission • Review medications • Consider thymectomy before pregnancy

40. Antenatal management • Team: OB/GYN + Neurologist + …… • Frequent hospitalization • Rest: Physical - Mental • Medications: - Pyridostigmine:  (Class C) - Corticosteroids: especially if in remission - Immunosuppressants: Azathioprine  , Others  - IV Ig  • Plasmapharesis:  BUT avoid ↓ BP & ↓ volemia • Drugs to be avoided: Narcotics, Muscle relaxants, MgSO4, Aminoglycosides • Fetal surveillance

42. Management during delivery • Time: according to obstetric indication • Route: according to obstetric indication • Oxytocin: allowed • Epidural & Regional analgesia: allowed • Drugs to be avoided: Narcotics, Muscle relaxants for GA, MgSO4, Aminoglycosides • 2nd stage: Forceps

44. Postpartum management • Revise medications: doses • Newborn: - 85% have antibodies - 10 – 20% have symptoms • Weak cry, poor suckling and respiratory distress • Temporary: resolves within 2 to 6 weeks • Treatment: parenteral neostigmine or small doses of edrophonium

46. Trivia Celebreties diagnosed with MG

47. Trivia “ Celebreties diagnosed with MG”

48. Trivia “ Celebreties diagnosed with MG” • Aristotle Onassis (Business man)

50. Trivia “ Celebreties diagnosed with MG” • Paris Hilton (Actress)

52. Trivia “ Celebreties diagnosed with MG” • Amitabh Bachchan (Actor)

54. Trivia “ Celebreties diagnosed with MG” • Sleepy (Cartoon character)

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