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Problemas terapéuticos en la Miastenia Gravis

Problemas terapéuticos en la Miastenia Gravis. Dra. Beatriz Arciere. MGFA Clinical Class Class I : Any ocular muscle weakness; may have weakness of eye closure. All other muscle strength is normal.

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Problemas terapéuticos en la Miastenia Gravis

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  1. Problemas terapéuticos en la Miastenia Gravis Dra. Beatriz Arciere

  2. MGFA Clinical Class • Class I: Any ocular muscle weakness; may have weakness of eye closure. All other muscle strength is normal. • Class II: Mild weakness affecting muscles other than ocular muscles; may also have ocular muscle weakness of any severity. • IIa. Weakness affecting limb muscles, axial muscles, or both. May also have lesser but not predominant involvement of oropharyngeal muscles. • IIb. Predominantly affecting oropharyngeal, respiratory muscles, or both. May also have lesser or equal involvement of limb, axial muscles, or both. • Class III: Moderate weakness affecting muscles other than ocular muscles; may also have ocular muscle weakness of any severity. • IIIa. Weakness affecting limb muscles, axial muscles, or both. May also have lesser but not predominant involvement of oropharyngeal muscles. • IIIb. Predominantly affecting oropharyngeal, respiratory muscles, or both. May also have lesser or equal involvement of limb axial muscles, or both. • Class IV: Severe weakness affecting muscles other than ocular muscles; may also have ocular muscle weakness of any severity. • IVa. Weakness affecting limb muscles, axial muscles, or both. May also have lesser but not predominant involvement of oropharyngeal muscles. • IVb. Predominantly affecting oropharyngeal, respiratory muscles, or both. May also have lesser or equal involvement of limb, axial muscles, or both. • Class V: Defined as intubation, with or without mechanical ventilation, except when employed during routine postoperative management. The use of a feeding tube without intubation places the patient in class IVb.

  3. Tratamiento • Incremento de la acetilcolina. B. de Piridostigmina • Decremento de Ac contra el receptor de acetilcolina. Prednisona Otros inmunosupresores: Azatioprina, ciclosporina, micofenolato de mofetilo, ciclofosfamida. Timectomía Plasmaféresis/IgG IV

  4. ¿Qué inmunosupresor elegir al inicio? • 1-Palace J and cols. A randomized double-blind trial of prednisolone alone or with azathioprine in myasthenia gravis.Myasthenia Gravis study group. Neurology 1998;50:1778-1783. • 2- Meriggioli MN and cols. Mycophenolate mofetil for myasthenia gravis: An analysis of efficacy, safety and tolerability. Neurology 2003; 61: 1438-1440. • 3- Koski CL. Intravenous immunoglobulin use during induction of immunosuppression in patients with Myasthenia Gravis. Neurology 2003; 60(Supplement 1):A 419.

  5. ¿Timectomía sí o no? • 1- Gronseth GS and col. Practice parameter : thymectomy for autoimmune myasthenia gravis (an evidence-based review) report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2000; 55:7-15.

  6. Tratamiento de la crisis miasténica • Definición.- • Tratamiento: • manejo general • manejo ventilatorio (CVF< 15ml/kg; NIF<20cmH2O; PEF<40 cmH2O) • mejora del bloqueo NM 1-Qureshi Al and cols. Plasma exchange versus intravenous immunoglobulin treatment in myasthenic crisis. Neurology 1999; 52: 629-632. 2- Rabinstein A and col. BiPAP in acute respiratory failure due to myasthenic crisis may prevent intubation. Neurology 2002; 59: 1647-1649.

  7. Gracias

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