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Myasthenia Gravis during pregnancy
Ahmed Alaa el dinAssistant Lecturer of OB/GYN
High Risk Pregnancy UnitCairo University
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
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
What is Myasthenia Gravis?
• MYO = MUSCLE• ASTHENIA = WEAKNESS• GRAVIS = SEVERE
Myasthenia Gravis =
Severe Muscle Weakness
What is Myasthenia Gravis?
• MG is an autoimmune neuromuscular disease leading to fluctuating muscle weakness and fatigue.
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
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
Etiology
• Autoimmune:IgG vs Acetyl choline receptors (AChR)diminished end-plate action potential weak muscle contractions
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
Clinical presentation
• The characteristic feature is Fatigability• Initially intermittent symptoms: EYE• Within 1 year: Generalized (85%)• Remissions and Exacerbations
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
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
Examination
• Muscle strength: Weakness• Deep tendon reflexes: Normal• Associated autoimmune diseases: SLE,
RA, DM, Thyroid
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
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
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
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
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
Life threatening presentations
Crises may be precipitated by:• Systemic diseases• Concurrent infections • Emotional upset
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.
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
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)
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 crisisOR
Before surgery
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
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
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
Effect of pregnancy on MG
• Unpredictable: Worsens, improves or unchanged.
• Risks: Exacerbation, Crises, Respiratory complications necessitating ventilation, Death
• Most dangerous in 1st ∆
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
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%)
Myasthenia Gravis AND Pregnancy
• Effect of pregnancy on MG.• Effect of MG on pregnancy.
• Pre-conceptional management.• Antenatal management.• Management during delivery.• Postpartum management
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
Myasthenia Gravis AND Pregnancy
• Effect of pregnancy on MG.• Effect of MG on pregnancy.
• Pre-conceptional management.• Antenatal management.• Management during delivery.• Postpartum management
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
Myasthenia Gravis AND Pregnancy
• Effect of pregnancy on MG.• Effect of MG on pregnancy.
• Pre-conceptional management.• Antenatal management.• Management during delivery.• Postpartum management
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
Myasthenia Gravis AND Pregnancy
• Effect of pregnancy on MG.• Effect of MG on pregnancy.
• Pre-conceptional management.• Antenatal management.• Management during delivery.• Postpartum management
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
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