Download Problemas terapéuticos en la MG - Sociedad de Neurología del

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.
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.
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
¿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: 14381440.
• 3- Koski CL. Intravenous immunoglobulin use
during induction of immunosuppression in patients
with Myasthenia Gravis. Neurology 2003;
60(Supplement 1):A 419.
¿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; 55:715.
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.
Gracias