terri kueber, crna, ms. explain the pathophysiology and classification of myasthenia gravis (mg)...
TRANSCRIPT
Terri Kueber, CRNA, MS
Myasthenia Gravis
Explain the pathophysiology and classification of Myasthenia Gravis (MG)
List the signs and symptoms and clinical manifestation
Describe the various treatments available for Myasthenia Gravis
Discuss the anesthetic considerations for patients with Myasthenia Gravis
Objectives
Myasthenia Gravis is an autoimmune neuromuscular
Consider a “Snowflake Disease”Cause is still unknownNo known cureHistory Incident and prevalence is increasing with 5-10
per 100,000 25,000 cases in USStill considered sporadicWomen versus menOften associated with other autoimmune disorders
Introduction
Class I: Ocular weakness, other muscle are normal
Class II: Mild weakness affecting other muscle
Class IIa: Muscles weakness affecting limbs and possibly oropharyngeal muscles
Class III: Moderate weakness/severe ocular weakness
Class IV: Severe weakness affecting limb muscle
Class V: Defined by intubation
MGFA/Osserman Classification
Ocular Myasthenia GravisClassic General Myasthenia GravisTransient Neonatal Myasthenia GravisJuvenile Myasthenia GravisCongenital TypesDrug-induced (Pencillamine, NDMR,
aminoglycosides, procainamide)
Pathophysiologic Type
Presenting Symptoms
Bilateral or Unilateral Ptosis
DiplopiaHoarsenessAltered SpeechProblems chewingDysphagiaLimited Facial
Expressions
Endrophonium (tensilon) Chloride Test2.6-3.3 mg initial doseNeostigmine may be used in patients
who do not respond to tensilonAuto-antibodies in MG – receptor
binding antibodies are present in 80% of patients
Thymic hyperplasia is present in 70%
Diagnostic Procedures
Electromyography (EMG) TestingRepetitive Nerve Stimulation (RNS)
Single Fiber EMG (SFEMG)Ocular CoolingOther Testing
Diagnostic Procedures (continue)
Congenital Myasthenia Syndromes (not autoimmune)
Drug induced MGEaton-Lambert Syndrome (related to Small
cell carcinoma of the lung)Hyperthyroidism (increases MG symptoms)Graves diseaseBotulismOphthalmoplegiaIntracranial pathology
Differential Diagnosis
Fatigue or insufficient sleep Stress, anxiety, illness Overexertion, repetitive motion Pain or depression Low potassium or thyroid levels Alcoholic beverages Sudden fear, or extreme anger Extreme temperatures Humidity Sunlight or bright lights Hot foods or beverages Medications (beta blockers, antibiotics, calcium blockers) Chemicals such as household cleaner, insecticides, pet sprays,
lawn chemicals
Factors that Worsen MG
Cholinesterase inhibitorPyridostigmine – most commonly usedNeostigmine – used less frequently
ThymectomyPlasmapheresisCorticoid Steroid TherapyImmunosuppressive therapy (azathioprine,
cyclosporine, etc.)Short-term Immunotherapy (IV
immunoglobulin)
Treatment for Myasthenia Gravis
Current health and symptomsPMH/PSHNMBA – MonitoringPost operative planPain controlInduction and Extubation criteria
Anesthetic Comsiderations
46 year old male diagnosis with MG in 6/2006Presenting symptoms: fatigue and weakness after
working out with intermittent dipopliaAt time of diagnosis weakness lead to respiratory
failure leading ventilation and respiratory support x 3 days
Residual symptoms: weakness, pain and left ptosis
Refractory to medical support under went a thymectomy in October 2009
Case Discussion #1
Height/Weight: 75”/95=4.7PFT’s FVC=65%, FEV1/FVC=61%Did not take his Pyridostigmine this am as he
was instructed not to take it (not by anesthesia department)
Case Discussion (Continued)
Gave Pyridostigmine 90 mg preoperatively IVAdditional dose give at 1400 in recovery roomInduction included: slow induction no
neuromuscular blocking agentsTotal Agents included: Propofol 400mg
Fentanyl 300Morphine 4mgMidazolam 3 mg
Intubation with glidescope secondary to NIM’s tube placement.
What did we do??
Successfully extubated at end of case
Received pyridostigmine in recovery room
ICU overnight
Discharge next day.
Patient Outcome
63 year old maleDiagnosis between 12/05 and 2/06Schedule for Laparoscopic Nissen
FundoplicationMultiple drug allergiesEx-smoker (2 PPD x 30 years)H/O: MG controlled with medications, HTN, diet
controlled, AST – normal with EF 55-65%Hiatal Hernia/Barrett’s EsophagusPrevious surgery included: Brow lift,
Laparoscopic Cholecystectomy
Case Discussion #2
Previous anesthetic recordsSurgeons desire muscle relaxationWe did:
Propofol 350mgFentanyl 400 mcgMidazolam 2 mgZofran/DecadronEphedrine 15mgVasopressin 2 units
Patient’s was successfully extubated in OR and discharge home the next day
How did the case finish??
Questions??
Thank you for your attention