_gravis重症肌无力课件

Myasthenia Gravis Mike Willcut Myasthenia What? w My-as-thee-nee-ah Grr-a-vis w Auto immune disease w Hereditary genetic mutation w Causes muscle weakness Where have I heard that? w Contraindication for w Versed w Fentanyl Precaution for sux Pathophysiology w Auto Immune w Antibodies attack Ach Receptors Decreased cell excitement o Electrical threshold o No action potential o Weakness Cell with MG This might be on the Quiz How does MG affect the body? w w A Blocks motor receptors A Blocks motor receptors causing weaknesscausing weakness w B - Increases stimulus threshold w C - Decreases stimulus threshold w D - Reduces amount of Ach released causing weakness Classification w1: Eye muscle weakness (EMW) w2: EMW and other mild muscle weakness a.Predominantly limb or axial muscles b.Predominantly bulbar or respiratory muscles w3: EMW and other moderate weakness a.Predominantly limb or axial muscles b.Predominantly bulbar or respiratory muscles w4: EMW and other severe weakness a.Predominantly limb or axial muscles b.Predominantly bulbar or respiratory muscles w5: Intubation needed to maintain airway Whos at Risk w Females 20-40 w Males 60-80 w Those with another AI Disease w Penicillamine users (rheumatoid med) w Enlarged Thymus or thymoma w 20 in 100,000 or 2 in 1million (discrepancy) Diseases Typically Associated With MG Diabetes Type 1 w Rheumatoid Arthritis w Lupus w Demyelination CNS Disease w Other Auto immune diseases What will we see? w Hallmark S/S w Eye lid drooping (ptosis) w Impaired speech (dysarthria) w Difficulty Swallowing (dysphagia) w Double Vision (dipoplia) w Easily fatigued, quick recovery with rest w Waddling gait Physical Exam w Test for muscle fatigueability w Look up and to the side for 30 sec w If supine, look at feet for 1min w Arms stretched forward for 1min w 10 deep knee bends or squats Look for ptosis and ask about diplopia Myasthenia Crisis Requires immediate assistance Shortness of breath Extreme muscle weakness General fatigueablility Respiratory muscle paralysis MG in Pregnancy w Many mothers get worse 1st trimester and better 2nd and 3rd trimester. w Up to 10% of infants born to mothers with MG will have Transient Neonatal Myasthenia. w Weak cry, poor muscle tone, difficulty breathing etc Interventions w Depending on severity w RSI w Supportive care Triggers w May be triggered by: w Infection w Stress w Fever w Adverse reaction to medications Hospital Tests w Blood tests w Tests for specific antibodies w Only 50% accurate w CT w Chest X-Ray w Endrophonium Test w IV injection of acetylcholinesterase inhibitor Treatment w Actylcholinesterase inhibitors w Corticosteroids immunosuppressants w Thymectomy w Plasmapheresis w Like dialysis Naturopathic Treatment w Counseling sessions w Eliminate stress and poor self image w Diet and nutrition w Moshe Daniel ND Prognosis w Very Good! w Most get relief from simple treatment w Some go into remission Only 2-3% mortality rate Fun Facts w Radix family of Chinese herbs can help with symptoms. w Gravitax, a combination of iron, aloe vera, and apiaceae w First discription of MG in 1672 w 1935 Prostigmin Demonstration for MG Sources wDaniel, Moshe. “The Treatment and Philosophy of Healing For Myasthenia Gravis .“ 29 Sep 2007 12 Dec 2008 . wHoward, James, F. “About Myasthenia Gravis.“ For Health Care Professionals 30 Sep 2006 12 Dec 2008 .