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46 Cards in this Set
- Front
- Back
Symptoms that are associated with myasthenic crisis are associated with:
a. myosis b. increased lacrimination c. increased salivation d. flaccid paralysis |
d. flaccid paralysis
-cholinergic crisis is associated with myosis and increased lacrimination and salivation |
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The most effective treatment for myasthenia crisis includes:
a. prednisolone b. plasmaphreresis c. atropine d. thymectomy |
b. plasmapheresis
-neostigmine or pyridostigmine are also used for management of a MG crisis but steroids and surgery are indicated for chronic management. Atropine is used in the tx. of a cholinergic crisis |
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Thymomas are most often associated with which of the following conditions?
a. hyperthyroidism b. anemia c. small cell carcinoma d. myasthenia gravis |
d. myasthenia gravis
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What is Myasthenia gravis?
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is an antibody-mediated autoimmune disease of the NMJ, specifically the "postsynaptic membrane"
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What immunoglobulin plays a role in the pathophysiology of MG.
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IgG
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What receptor site does IgG block at the NMJ? And what % of patients with MG is IgG present in?
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Blocks the ACh binding sites on the nAChR (nicotinic acetylcholine receptor) and ultimately destroy's them.
present in approximately 80-85% |
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What % of MG patients also have a thymus disorder?
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75%
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What other diseases are associated with MG?
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-thyroid disease
-RA -Ulcerative colitis -Pernicious anemia -Lupus -Vitiligo |
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Symptoms of MG are usually prevalent when approx. ______ % of receptors remain
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30%
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What are the most common symptoms of MG.
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Ptosis & diplopia
Others: (bulbar muscles) difficulty swallowing, chewing, speech; generalized weakness; fatigue on repeated effort |
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What muscle group is affected in a pt with MG crisis?
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bulbar muscles (difficulty swallowing, chewing, speech)
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For Dx of MG, what is present in 85% of patients?
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Anti AChR antibodies
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What is the standard test used to diagnose MG
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Tensilon test
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how does the tension test effect:
A patient with MG A patient with cholinergic crisis |
MG- will show improvement
CC- Increased SLUDGE |
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What does "SLUDGE" stand for?
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S = salivation
L = Lacrimation U = Urination D = Diarrhea G = GI upset E = Emesis |
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Classification of MG involves Grade 1 - 4.
Grade 1 = Grade 2a = Grade 2b = Grade 3 = Grade 4 = |
Grade 1 = affects eyes only
Grade 2a = mild generalized disease; responds well to therapy. Grade 2b = Moderately severe disease Grade 3 = Severe generalized disease Grade 4 = Myasthenic Crisis (requires ventilation) |
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What is Myasthenic Crisis?
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-insufficient medications (under-dosing anti cholinesterase drugs can result in Myasthenic crisis)
-Inability to support head -Jaw is slack, body is limp -loss of gag reflex -*aspiration potential |
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What is Cholinergic Crisis?
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-can have similar presentation to MG
-Too much medication (can produce a surplus of ACh at the NMJ causing a depolarizing-like block) - Flaccid paralysis - Muscarinic side effects (SLUDGE) |
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What side effects predominate in the patient with Cholinergic Crisis?
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Muscarinic side effects (SLUDGE)
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What Medical treatment produces an 80% remission rate in MG
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Corticosteriod therapy
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How do cholinesterase inhibitors reduce the symptoms of MG
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by inhibiting the hydrolysis of ACh and therefore increasing the neurotransmitter's concentration at the NMJ
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What is the most commonly use anti cholinesterase agent
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pyridostigmine
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What is the treatment of choice in patients with more debilitating, widespread disease?
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immunosuppresive agents`
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Where are the Anti-AChR antibodies produced?
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thymus gland
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How will a surgical "Thymectomy" be beneficial for a MG patient
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it effectively arrests or reverses the myasthenia process by removing a major source of antibody production
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What is the Goal of "thymectomy"
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Goal is to reduce the amount of immunosuppressants used
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What is considered a short-term treatment for MG and stops severe "refractive MG" by reducing the concentration of circulating antibodies
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plasmapheresis
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Approx. what % of patients with MG have a Thymoma?
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10-15%
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What is the concern with Thymoma's
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-may cause airway or vascular obstruction post indcution
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What are some preoperative considerations for a pt with MG
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-assess respiratory fx.
- consider plasmapheresis & stopping immunosuppressants - Consider Regional (safest) |
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Why is the use of succinylcholine contraindicated in patients with MG?
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- resistance to Succ...D/T loss of ACh receptors.
- ED 95 is 2.6 x normal - They develop phase II block |
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Why is the use of NDNMB contraindicated in patients with MG?
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-Have increased sensitivity to NMB
-So, use short acting agents - titrate small doses -avoid pre-treatment |
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What IV anesthetic agents typically have no adverse effect on patients with MG?
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Barbiturates, propofol, etomidate, ketamine
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What is the concern with opioids in patients with MG?
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May cause respiratory depression - b/c of already weak respiratory muscles....
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What drugs are known to depress NM transmission in MG patients?
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aminoglycosides, polymyxins
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What drugs are known to exacerbate MG?
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B-blockers, corticosteroids
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How do steroids affect Succ and NDMR?
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-do not affect dose response to succ.
-but, decrease dose requirements for NDMR |
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What 2 drugs are known to cause weakness in patients with MG?
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Procainamide & Phenytoin
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should you reverse a patient who has MG prior to extubation?
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They may be ineffective - so avoid if possible, just allow them to wear off
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What % of MG patients require PO ventilation?
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50%...They may have good peripheral strength but weak respiratory muscles
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What are the risk factors for "PO Ventilation"
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- FVC < 2.9 L
-MG > 6 years -Grade III or IV MG - Pyridostigmine dose > 750mg/day - Major surgery -coexisting lung disease |
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What is the difference between MG and LEMS (lambert-Eaton myasthenic syndrome)?
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MG associated with post-synaptic nAChR antibodies,
-LEMS is associated with autoantibodies that antagonize the function of the presynaptic ACh. Ca+ channels..."restricting the release of ACh" |
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_________, is characteristic of LEMS
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decreased ACh release
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The symptoms of Eaton-Lambert's is worse during which part of the day?
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usually worse in morning and improve during day
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Which part of the body is affected most in LEMS?
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associated with muscle weakness in the "proximal limbs"
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Eaton-Lambert Syndrome is often associated with what?
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with neoplasm (SCLC - small cell lung carcinoma)
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