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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
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
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
What is Myasthenia gravis?
is an antibody-mediated autoimmune disease of the NMJ, specifically the "postsynaptic membrane"
What immunoglobulin plays a role in the pathophysiology of MG.
IgG
What receptor site does IgG block at the NMJ? And what % of patients with MG is IgG present in?
Blocks the ACh binding sites on the nAChR (nicotinic acetylcholine receptor) and ultimately destroy's them.

present in approximately 80-85%
What % of MG patients also have a thymus disorder?
75%
What other diseases are associated with MG?
-thyroid disease
-RA
-Ulcerative colitis
-Pernicious anemia
-Lupus
-Vitiligo
Symptoms of MG are usually prevalent when approx. ______ % of receptors remain
30%
What are the most common symptoms of MG.
Ptosis & diplopia

Others: (bulbar muscles) difficulty swallowing, chewing, speech; generalized weakness; fatigue on repeated effort
What muscle group is affected in a pt with MG crisis?
bulbar muscles (difficulty swallowing, chewing, speech)
For Dx of MG, what is present in 85% of patients?
Anti AChR antibodies
What is the standard test used to diagnose MG
Tensilon test
how does the tension test effect:

A patient with MG

A patient with cholinergic crisis
MG- will show improvement

CC- Increased SLUDGE
What does "SLUDGE" stand for?
S = salivation
L = Lacrimation
U = Urination
D = Diarrhea
G = GI upset
E = Emesis
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)
What is Myasthenic Crisis?
-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
What is Cholinergic Crisis?
-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)
What side effects predominate in the patient with Cholinergic Crisis?
Muscarinic side effects (SLUDGE)
What Medical treatment produces an 80% remission rate in MG
Corticosteriod therapy
How do cholinesterase inhibitors reduce the symptoms of MG
by inhibiting the hydrolysis of ACh and therefore increasing the neurotransmitter's concentration at the NMJ
What is the most commonly use anti cholinesterase agent
pyridostigmine
What is the treatment of choice in patients with more debilitating, widespread disease?
immunosuppresive agents`
Where are the Anti-AChR antibodies produced?
thymus gland
How will a surgical "Thymectomy" be beneficial for a MG patient
it effectively arrests or reverses the myasthenia process by removing a major source of antibody production
What is the Goal of "thymectomy"
Goal is to reduce the amount of immunosuppressants used
What is considered a short-term treatment for MG and stops severe "refractive MG" by reducing the concentration of circulating antibodies
plasmapheresis
Approx. what % of patients with MG have a Thymoma?
10-15%
What is the concern with Thymoma's
-may cause airway or vascular obstruction post indcution
What are some preoperative considerations for a pt with MG
-assess respiratory fx.

- consider plasmapheresis & stopping immunosuppressants

- Consider Regional (safest)
Why is the use of succinylcholine contraindicated in patients with MG?
- resistance to Succ...D/T loss of ACh receptors.

- ED 95 is 2.6 x normal

- They develop phase II block
Why is the use of NDNMB contraindicated in patients with MG?
-Have increased sensitivity to NMB

-So, use short acting agents
- titrate small doses
-avoid pre-treatment
What IV anesthetic agents typically have no adverse effect on patients with MG?
Barbiturates, propofol, etomidate, ketamine
What is the concern with opioids in patients with MG?
May cause respiratory depression - b/c of already weak respiratory muscles....
What drugs are known to depress NM transmission in MG patients?
aminoglycosides, polymyxins
What drugs are known to exacerbate MG?
B-blockers, corticosteroids
How do steroids affect Succ and NDMR?
-do not affect dose response to succ.

-but, decrease dose requirements for NDMR
What 2 drugs are known to cause weakness in patients with MG?
Procainamide & Phenytoin
should you reverse a patient who has MG prior to extubation?
They may be ineffective - so avoid if possible, just allow them to wear off
What % of MG patients require PO ventilation?
50%...They may have good peripheral strength but weak respiratory muscles
What are the risk factors for "PO Ventilation"
- FVC < 2.9 L

-MG > 6 years

-Grade III or IV MG

- Pyridostigmine dose > 750mg/day

- Major surgery

-coexisting lung disease
What is the difference between MG and LEMS (lambert-Eaton myasthenic syndrome)?
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"
_________, is characteristic of LEMS
decreased ACh release
The symptoms of Eaton-Lambert's is worse during which part of the day?
usually worse in morning and improve during day
Which part of the body is affected most in LEMS?
associated with muscle weakness in the "proximal limbs"
Eaton-Lambert Syndrome is often associated with what?
with neoplasm (SCLC - small cell lung carcinoma)