Myasthenia Gravis：重癥肌無力

1、Myasthenia GravisMike WillcutMyasthenia What?w My-as-thee-nee-ah Grr-a-visw Auto immune diseasew Hereditary genetic mutationw Causes muscle weaknessWhere have I heard that?w Contraindication forw Versedw FentanylPrecaution for suxPathophysiologyw Auto Immunew Antibodies attack Ach ReceptorsDecreased

2、 cell excitemento Electrical thresholdo No action potentialo WeaknessCell with MG This might be on the QuizHow does MG affect the body?w B - Increases stimulus thresholdw C - Decreases stimulus thresholdw D - Reduces amount of Ach released causing weaknessClassificationw1: Eye muscle weakness (EMW)w

3、2: EMW and other mild muscle weaknessa.Predominantly limb or axial musclesb.Predominantly bulbar or respiratory musclesw3: EMW and other moderate weaknessa.Predominantly limb or axial musclesb.Predominantly bulbar or respiratory musclesw4: EMW and other severe weaknessa.Predominantly limb or axial m

4、usclesb.Predominantly bulbar or respiratory musclesw5: Intubation needed to maintain airwayWhos at Riskw Females 20-40w Males 60-80w Those with another AI Diseasew Penicillamine users (rheumatoid med)w Enlarged Thymus or thymomaw 20 in 100,000 or 2 in 1million (discrepancy)Diseases Typically Associa

5、ted With MG Diabetes Type 1w Rheumatoid Arthritisw Lupusw Demyelination CNS Diseasew Other Auto immune diseasesWhat will we see?w Hallmark S/Sw Eye lid drooping (ptosis)w Impaired speech (dysarthria) w Difficulty Swallowing (dysphagia)w Double Vision (dipoplia) w Easily fatigued, quick recovery with

6、 restw Waddling gaitPhysical Examw Test for muscle fatigueabilityw Look up and to the side for 30 secw If supine, look at feet for 1minw Arms stretched forward for 1minw 10 deep knee bends or squats Look for ptosis and ask about diplopiaMyasthenia CrisisRequires immediate assistance Shortness of bre

7、ath Extreme muscle weakness General fatigueablility Respiratory muscle paralysisMG in Pregnancyw 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 breat

8、hing etcInterventionsw Depending on severityw RSIw Supportive careTriggersw May be triggered by:w Infectionw Stressw Feverw Adverse reaction to medicationsHospital Testsw Blood testsw Tests for specific antibodiesw Only 50% accuratew CTw Chest X-Rayw Endrophonium Testw IV injection of acetylcholines

9、terase inhibitor Treatmentw Actylcholinesterase inhibitorsw Corticosteroids immunosuppressantsw Thymectomyw Plasmapheresisw Like dialysisNaturopathic Treatmentw Counseling sessionsw Eliminate stress and poor self image w Diet and nutritionw Moshe Daniel NDPrognosisw Very Good!w Most get relief from

10、simple treatmentw Some go into remissionOnly 2-3% mortality rateFun Factsw Radix family of Chinese herbs can help with symptoms.w Gravitax, a combination of iron, aloe vera, and apiaceaew First discription of MG in 1672w 1935 Prostigmin Demonstration for MGSourceswDaniel, 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 . wMyast