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NMSP Exam 2 (for the Gunners) > Disorders of the NMJ > Flashcards

Disorders of the NMJ Flashcards

1
Q

What is MYasthenia Gravis?

A

Defect of neuromuscular transmission due to an antibody-mediated attack upon nicotinic acetylcholine receptors on muscle membrane

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2
Q

While most cases of MG are sporadic, what haplotypes are most associated?

A

HLA B8 and DR3

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3
Q

What other autoimmune disorders are associated with MG?

A

SLE, RA, thyroid disorders, etc.

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4
Q

In which populations is MG more common?

A

Young women and older men

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5
Q

What are the three general characteristics of MG?

A
  1. Fluctuating weakness
  2. Ocular muscle weakness, dysarthria, dysphagia, limb/neck weakness
  3. Respond to cholinergic drugs
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6
Q

What are the 4 classifications of MG?

A

I: Purely ocular

II: Moderately severe, generalized (most common)

III: Acute fulminating

IV: Late severe

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7
Q

What lab data will you see with MG?

A

Anti-AChR Abs

MUSK and LRP-4 AB

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8
Q

What EMG findings will you see with MG?

A
  • Decremental response on repetitive stimulation
  • Increased “Jitter” on single fiber EMG
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9
Q

What are some speciality or clinical tests done to assess for MG?

A

Tensilon (edrophonium test)-rarely done

Fatigable weakness/ice bag test, Cogan’s sign

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10
Q

What are some general treatments for MG?

A

Anticholinesterase drugs (Mestinon)

Prednisone

Immunosuppressants, IVIG

Thymectomy

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11
Q

What is myasthenic crisis?

What can trigger it?

What can it cause?

What is the treatment?

A

rapid deterioration of the disease itself

  • can be spontaneous after infections/drug use
  • can cause aspiration, diffuse weakness, resp. failure
  • treat by stoping anticholinesterase meds, use PLEX, IVIG, maybe steroids
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12
Q

What is cholinergic crisis?

A

rapid increase in weakness from excess anticholinesterase meds

  • Sx include N/V, sweating, salivation, colic, diarrhea, bradycardia
  • BIG CLUE: miosis and or fasciculations
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13
Q

What ab will be seen in Seronegtive MG?

What are the three types?

A

10% will have no anti-AChR Abs, and 40% of these will have MUSK abs

  1. Oculopharyngeal weakness
  2. Neck, shoulder, resp. weakness
  3. Indistinguishable from Ab + MG
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14
Q

What is the treatment for seronegative MG?

A

PLEX, IVIG, and Rituximab is best

Remission is possible

Poor response to anticholinesterase meds/thymectomy

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15
Q

What is Lambert-Eaton Myasthenic Syndrome?

A

Autoimmune attack against voltage-gated calcium channels on the presynaptic nerve terminal

Leads to weakness

Often associated with cancer (SCCL)

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16
Q

What is the clinical presentation of LEMS?

A

Proximal weakness, loss of DTRs, myalgias, dry mouth, impotence

Oropharyngeal/ocular muscles may be affected, but not like MG

Strength may improve after exercise

May have slight response to Tensilon test

17
Q

What Lab data is expected with LEMS?

A

Anti-VGCC Abs

18
Q

What are the EMG findings associated with LEMS?

A

Low amplitude motor response that facilitates after a brief period of exercise

Incremental response on fast repetitive stimulation

19
Q

What is the treatment for LEMS?

A
  1. treat malignancy
  2. Acetylcholinesterase inhibitors
  3. Amifampridine/3-4 Diaminopyridine/Gaunidine hydrochloride
  4. Immunosuppressents/IVIG
20
Q

What is the clinical presentation of Botulism?

A

Toxin blocks presynaptic release of Ach

Presents with dry mouth, blurred vision, N/V, hypohidrosis, muscle paralysis

21
Q

What is the treatment for botulism?

A

ICU monitoring with resp. support

Antitoxin or Guanidine Hydrochloride

22
Q

What are the symptoms of Sarin and VX nerve gases?

A

Inhibit Achesterase at NMJ to cause end organ overstimulation

Miosis, secretions, bronchospasm, cramps, N/V/D, HR/BP changes, muscular weakness, SZs, LOC

Death by resp. failure

23
Q

What is the treatment for Sarin or VX gas?

A

Decontamination

Resp. support

Atropine 2-6mg q 5-10 min until secretions recede

2-Pralidoxime chloride: 1gm IV over 20-30 minues

Szs-benzos

NMSP Exam 2 (for the Gunners) (19 decks)

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