Myasthenia Gravis is an autoimmune neuromuscular disease caused by antibodies against acetylcholine receptors. It is classified based on the severity and distribution of symptoms, ranging from purely ocular (Class I) to severe generalized weakness (Class III/IV). Abnormal thymus glands are present in 75% of patients and thymectomy often improves symptoms. Treatment includes anticholinesterase medications, immunosuppressants, plasmapheresis, and management of comorbid conditions. Anesthesia management focuses on timing of anticholinesterase doses, potential for respiratory complications, and extubation criteria involving strength and pulmonary function tests.
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Myasthenia Gravis
Myasthenia Gravis is an autoimmune neuromuscular disease caused by antibodies against acetylcholine receptors. It is classified based on the severity and distribution of symptoms, ranging from purely ocular (Class I) to severe generalized weakness (Class III/IV). Abnormal thymus glands are present in 75% of patients and thymectomy often improves symptoms. Treatment includes anticholinesterase medications, immunosuppressants, plasmapheresis, and management of comorbid conditions. Anesthesia management focuses on timing of anticholinesterase doses, potential for respiratory complications, and extubation criteria involving strength and pulmonary function tests.
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Myasthenia Gravis
Osserman and Genkins classification:
• Class I—ocular symptoms only • Class IA—ocular S’s with EMG evidence of peripheral muscle involvement • Class IIA—mild generalized symptoms • Class IIB—more severe and rapidly progressive symptoms • Class III—acute, presenting in weeks to months with severe bulbar symptoms • Class IV—late in the course of the disease with severe bulbar symptoms and marked generalized weakness • autoimmune disease with anti-acetylcholine receptor antibodies, F>M • Abnormal thymus glands 75% of pts(85% show hyperplasia; 15% thymoma). 75% of pts either go into remission or are improved post-op • Medical ttt: anticholinesterase, steroids, other immunosuppressant (azathioprine, cyclophosphamide, cyclosporine)and plasmapheresis. • underdosage → “myasthenic crisis” whereas overdosage will produce a “cholinergic crisis.” Excessive doses of cholinesterase inhibitors produce abdominal cramping, vomiting, diarrhea, salivation, bradycardia, and skeletal muscle weakness that mimics the weakness of myasthenia , to differentiate between them→”Tensilon test” 2-10 mg IV Edrophonium→ with M.crisis they improve, but not with C.crisis. • Sensitive to NDMR, resistant to Sux→ ↑ dose to 1.5 mg/kg Anesthesia Management • best to be done 1st case during the day, avoid pre-med • Best to hold the AM dose of anticholinesterase, unless the Pt is physically and/or psych dep A→ possible mediastinal mass, may need RSI, possible Difficult with other diseases B→ frequent aspiration, resp failure → PFT C→ focal myocarditis, A.fib , AV block D→ steroids (stress dose), immunosuppressant, anticholinesterase(dose), avoid drugs that may potentiate NMB (aminoglycoside ABx, quinidine, CCB) H→ anemia, ITP, lymphoma, leukemia CNS→ MS M→ thyroid dysfunction ↑ or ↓, Other→ R. arthritis, SLE, scleroderma Lab→ CBC, lytes (abnormality may ↑ weakness), PFT, ECG, chest CT, CXR. • Monitor N. stimulator. • Post-op problems : pneumonia due to poor coughing, Aspiration, resp failure Leventhal, assigned a scoring system to four factors they found to be predictive for requirement of post-op mechanical vent (for transternal thymectomy) • Duration of >6 years 12 points • History of chronic obstructive pulmonary disease 10 points • >750 mg/d pyridostigmine 8 points • Vital capacity <2.9 liters 4 points Patients scoring <10 points in their series could be extubated immediately postoperatively; those scoring >12 points required postoperative ventilatory support. • Post-op pain Mx best using regional anesthesia • Have an ICU backup bed ready. • Extubation criteria o Awake and responsive, stable V/S, good grip, sustained head left o Good ABG, on FiO2 < 40%(>90%sat) , with adequate vent and oxy maintained by the Pt o –ve inspiratory pressure > -20cmH2O o VC > 15ml/kg • Pregnancy may cause exacerbation or remission, with ↑ remission postpartum • The neonate may have transient MG