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Miastenia Gravis actualización Dr. Juan Lozano
Thomas Willis (1621-1675), English physician, published a book,  De anima brutorum (1) in  1672 in which he wrote about "a woman who temporarily lost her power of speech  and became 'mute as a fish. '"(1) This has been interpreted as being the first written description of myasthenia gravis.  Thomas Willis was born in Great Bedwin, a  Wiltshire village in England. He graduated from Oxford University  Legion with a Bachelor of Medicine in 1646.  In 1660 he was appointed  Sedleian Professor of Natural Philosophy at Oxford and given the M.D.(2)
Her first patient, 'Mrs. M.', had had intermittent weakness for 14 years and had been admitted to St. Alfege's Hospital about 2 months prior to the treatment given by Dr. Walker because of an exacerbation. Mrs. M's muscle power was restored about 30 mins after the hypodermic injection of physostigmine sulphate (gr. 1/60) --- the effect lasted up to 4 hours. The case was written up in the Lancet 2nd June 1934, pp. 1200-1 and was also reported in the local newspaper The Kentish Mercury on 15th March 1935. Dra. Mary Walker
Paciente de la Dra Walker 'Mrs. M.' before (left image) and after (right image) injection of physostigmine.  Before - patient cannot raise left eyelid. After - eye is fully open. These photos were reproduced from a cine film and appeared in reverse (right for left) in The Lancet 1934 (i) 1200-1. They are reproduced here with correct orientation.
GENTE FAMOSA CON MG ,[object Object],[object Object],[object Object],[object Object],[object Object]
 
Julie Long artista Jerry lewis
Epidemiología ,[object Object],[object Object],[object Object]
Miastenia Gravis ,[object Object],[object Object]
Miastenia Gravis ,[object Object],[object Object],[object Object],[object Object],[object Object]
Unión  neuromuscular
Edad de inicio 2a. y 3a. década en la mujer. 7a. y 8a. década en el hombre Los hombres son mas afectados que las mujeres.
Miastenia Gravis Datos Clínicos ,[object Object],[object Object]
Miastenia Gravis ,[object Object]
 
Síntomas Iniciales ,[object Object],[object Object],[object Object]
Miastenia Gravis ,[object Object]
 
 
 
 
DEBILIDAD FATIGABLE
 
Crisis Miasténica ,[object Object],[object Object],[object Object]
 
Procedimientos Diagnósticos Prueba del Tensilón ,[object Object],[object Object],[object Object],[object Object]
 
Prueba de Tensilón ,[object Object],[object Object],[object Object],[object Object]
Despues de tensilón Prueba de tensilón Antes de tensilón
Anticuerpos Antireceptores de acetilcolina ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Procedimientos Diagnósticos Electromiografía. ,[object Object],[object Object]
Clasificación Clínica ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
 
 
Steinert Kearn-sayre
tratamiento ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Dosis equivalentes de anticolinesterasicos   Dosis (mg) y vía Oral IM IV Jarabe Neostigmina 15 Neostigmina 1.5 0.5 Mestinon 60 2 0.7 60 mg 5 ml Mestinon Timespan 90 - 180 Ambenomio 7.5
Anticolinesterásicos toxicidad y efectos secundarios ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Glucocorticoides Efectos secundarios a largo plazo Gastrointestinales Nausea,vómitos,  Gastritis, ulceras Dermatologicos ac né, hirsutismo Cushing Endocrino s: Hiperglucemia Hipokalemia . Inmunológicos : Inmunodepresión. Hematológicos : petequias Cardiovascular HAS edema Oftalmológicos : Cataratas Glaucoma Psicológicos: Depresión Insomnio. Otros: Aumento de peso
Azatioprina Efectos secundarios Gastrointestinales Nausea,vómitos,diarrea Ulceras,malestar Hematológicos Leucopenia, anemia Trombocitopenia. Hepático Enzimas Hepáticas elevadas Inmunológico Mayor susceptibilidad a infecciones Neoplásico Incrementa el riesgo para linfoma
Timectomia ,[object Object],[object Object],[object Object],[object Object],[object Object]
TIMOMA
Terapias emergentes ,[object Object],[object Object]
 
El que alguien toque mi vida es un privilegio,  Tocar la vida de alguien es un honor, Pero el ayudar a que otros toquen sus propias vidas Es un placer indescriptible!   Rubén Darío
 
 
 
 
 
Major Events in Neuromuscular Transmission ,[object Object],[object Object],[object Object],[object Object],[object Object]
Two main Types of Neuromuscular Blocking Drugs ,[object Object],[object Object]
Mechanism of Action of Nondepolarizing Neuromuscular Blocking Drugs
Non-depolarizing  (competitive). ,[object Object],[object Object],[object Object],[object Object]
Nondepolarizing Agents ,[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object]
Depolarizing Agents
Depolarizing Agents ,[object Object],[object Object],[object Object],[object Object],[object Object]
Depolarizing Agents ,[object Object],[object Object],[object Object]
Cholinesterase Inhibitors
Cholinesterase Inhibitors ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Myasthenia Gravis                                                
Myasthenia Gravis  is an autoimmune Disease that is characterized by a decrease in number of AChR Because there are fewer AChR to bind to the end plate potentials (EPPs) are smaller. With smaller EPPs the “ safety factor” is reduced there is less chance that the post-synaptic muscle fibres will be activated
Note: The amplitude of the end plate-potential is directly related to the amount of ACh that binds to the post-synaptic AChRs.
Myasthenia Gravis
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Malignant Hyperthermia  Dantrolene  (interferes with EC coupling by decreasing Ca exflux from the SR
Diazepam  (A  Benzodiazepine  that probably f acilitates the actions of GABA A  in the CNS) Baclofen  (GABA B   agonist – note error in your handouts) Primarily used in the treatment of spastiticy associated with spinal cord injury  Spasmolytic Drugs

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Miastenia gravis

  • 2. Thomas Willis (1621-1675), English physician, published a book, De anima brutorum (1) in 1672 in which he wrote about "a woman who temporarily lost her power of speech and became 'mute as a fish. '"(1) This has been interpreted as being the first written description of myasthenia gravis. Thomas Willis was born in Great Bedwin, a Wiltshire village in England. He graduated from Oxford University Legion with a Bachelor of Medicine in 1646. In 1660 he was appointed Sedleian Professor of Natural Philosophy at Oxford and given the M.D.(2)
  • 3. Her first patient, 'Mrs. M.', had had intermittent weakness for 14 years and had been admitted to St. Alfege's Hospital about 2 months prior to the treatment given by Dr. Walker because of an exacerbation. Mrs. M's muscle power was restored about 30 mins after the hypodermic injection of physostigmine sulphate (gr. 1/60) --- the effect lasted up to 4 hours. The case was written up in the Lancet 2nd June 1934, pp. 1200-1 and was also reported in the local newspaper The Kentish Mercury on 15th March 1935. Dra. Mary Walker
  • 4. Paciente de la Dra Walker 'Mrs. M.' before (left image) and after (right image) injection of physostigmine. Before - patient cannot raise left eyelid. After - eye is fully open. These photos were reproduced from a cine film and appeared in reverse (right for left) in The Lancet 1934 (i) 1200-1. They are reproduced here with correct orientation.
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  • 7. Julie Long artista Jerry lewis
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  • 12. Edad de inicio 2a. y 3a. década en la mujer. 7a. y 8a. década en el hombre Los hombres son mas afectados que las mujeres.
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  • 29. Despues de tensilón Prueba de tensilón Antes de tensilón
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  • 37. Dosis equivalentes de anticolinesterasicos Dosis (mg) y vía Oral IM IV Jarabe Neostigmina 15 Neostigmina 1.5 0.5 Mestinon 60 2 0.7 60 mg 5 ml Mestinon Timespan 90 - 180 Ambenomio 7.5
  • 38.
  • 39. Glucocorticoides Efectos secundarios a largo plazo Gastrointestinales Nausea,vómitos, Gastritis, ulceras Dermatologicos ac né, hirsutismo Cushing Endocrino s: Hiperglucemia Hipokalemia . Inmunológicos : Inmunodepresión. Hematológicos : petequias Cardiovascular HAS edema Oftalmológicos : Cataratas Glaucoma Psicológicos: Depresión Insomnio. Otros: Aumento de peso
  • 40. Azatioprina Efectos secundarios Gastrointestinales Nausea,vómitos,diarrea Ulceras,malestar Hematológicos Leucopenia, anemia Trombocitopenia. Hepático Enzimas Hepáticas elevadas Inmunológico Mayor susceptibilidad a infecciones Neoplásico Incrementa el riesgo para linfoma
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  • 45. El que alguien toque mi vida es un privilegio, Tocar la vida de alguien es un honor, Pero el ayudar a que otros toquen sus propias vidas Es un placer indescriptible!   Rubén Darío
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  • 53. Mechanism of Action of Nondepolarizing Neuromuscular Blocking Drugs
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  • 62. Myasthenia Gravis                                                
  • 63. Myasthenia Gravis is an autoimmune Disease that is characterized by a decrease in number of AChR Because there are fewer AChR to bind to the end plate potentials (EPPs) are smaller. With smaller EPPs the “ safety factor” is reduced there is less chance that the post-synaptic muscle fibres will be activated
  • 64. Note: The amplitude of the end plate-potential is directly related to the amount of ACh that binds to the post-synaptic AChRs.
  • 66.
  • 67. Malignant Hyperthermia Dantrolene (interferes with EC coupling by decreasing Ca exflux from the SR
  • 68. Diazepam (A Benzodiazepine that probably f acilitates the actions of GABA A in the CNS) Baclofen (GABA B agonist – note error in your handouts) Primarily used in the treatment of spastiticy associated with spinal cord injury Spasmolytic Drugs