Myasthenia Gravis and Multiple Sclerosis Flashcards

1
Q

what is the most common disorder of neuromuscular transmission

A
  • myasthenia gravis
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2
Q

what is myasthenia gravis caused by

A
  • autoimmune disorder which attacked acetylocholine receptors at the NMJ
    • causing weakness and fatigability of muscles
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3
Q

what are the two types of myasthenia gravis

A
  • ocular myasthenia gravis
  • generalized myasthenia gravis
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4
Q

myasthenia gravis is associated with what other conditions

A
  • other autoimmune conditions
    • RA, SLE, autoimmune thyroiditis
  • thymus abnormality (75%)
    • hyperplasia or thymoma
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5
Q

Myasthenia Gravis is hallmarked by weakness in what 4 things

A
  • ocular
  • bulbar (speech, chewing, swallowing)
  • limb (typically upper extremities)
  • respiratory muscles
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6
Q

what condition is characterized by fluctuating weakness and faitgue of specific. muscle groups. Weakness worse at end of day or after exercise.

A

myasthenia gravis

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7
Q

myasthenia gravis symptoms are worsened by

A
  • hot temperature
  • surgery
  • emotional stress
  • illness
  • menstruation
  • medication
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8
Q

List 3 medications that make myasthenia gravis worse

A
  • Fluoroquinolones
  • Beta blockers
  • Hydroxychloroquine
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9
Q

what is the bimodal distribution of ae for myasthenia gravis

A
  • 20s-30s (female > male)
  • 60s-80s (male > female)
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10
Q

what studies would you do to assess for myasthenia gravis

A
  • EMG and nerve conduction study
    • will see decreased response on repetitive nerve stimulation
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11
Q

What test can be done for myasthenia gravis

A
  • Edrophonium (Tensilon) test
    • inhibits the enzyme acetylcholinesterase and allows the Ach to stimulate the limited number of Ach receptors
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12
Q

what is a quick screening test that can be done to check for myasthenia gravis

A
  • Ice pack test
    • cold temperature improve neuromuscular transmission
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13
Q

what are the serologic tests for autoantibodies to make the myasthenia gravis diagnosis

A
  • AChR Ab: acetylcholine receptor antibodies
  • MuSK Ab: Muscle specific kinase antibodies
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14
Q

why would you order a CXR or CT chest if you suspect myasthenia gravis

A
  • chest for thymic abnormalities/thymoma
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15
Q

What is Lambert Eaton myasthenic syndrome

A
  • impaired release of Ach
  • often associated with small cell lung cancer
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16
Q

how can Lambert Eaton myasthenic syndrome be differentiated from Myasthenia Gravis

A
  • LE:
    • leg weakness common
    • sx’s worse in morning and improve with exercise
17
Q

what medication are patients with Myasthenia Gravis commonly put on treat symtpoms

A
  • Pyridostigmine bromide (Mestinon)
    • allows for prolonged effect of Ach strength
18
Q

Pyridostigmine bromide (Mestinon) is best for tx of which symptoms

A
  • limb and bulbar symtpoms
19
Q

What is Multiple Sclerosis

A
  • immune mediated inflammatory demyelinating disease of the CNS
  • attacks myelin sheaths of nerves, causing plaques of demyelination
20
Q

test of choice for Multiple Sclerosis

A
  • MRI
    • white matter changes - represent plaques within the myelin
21
Q

what is the hallmark characteristic of Multiple Sclerosis

A
  • symptomatic episodes that occur months to years apart and affect different anatomic locations
    • seperation of time and space
22
Q

Multiple Sclerosis usually affects what patient poplulation

A
  • age: 15-45
  • female > male
23
Q

List the 4 types of Multiple Sclerosis

A
  • Relapsing-remitting disease (RRMS)
  • Clinically isolated syndrome
  • Primary progressive (PPMS)
  • Secondary progressive (SPMS)
24
Q

what is the most common type of Multiple Sclerosis

A
  • relapsing-remitting disease
25
Q

describe relapsing-remitting disease

A
  • clearly defined relapses with full recovery or some deficit
26
Q

describe clinically isolated syndrome

A
  • 1st attack of MS symptoms
27
Q

differentiate between primary progressive MS and secondary progressive MS

A
  • primary progressive: progressive increase in symptoms from disease onset
  • secondary progressive: initially RRMS with gradual worsening
28
Q

typical CNS symtpoms associated with Multiple sclerosis

A
  • optic neuritis (eye pain leading to visual loss)
  • paresthesia
  • weakness
  • cerebellar: nystagmus, incoordination, tremor
  • Upper motor neuron signs
29
Q

Lhermitte’s sign? associated with?

A
  • neck flexion causes electrical shock sensation down back/limbs
  • Multiple sclerosis
30
Q

what is a marcus Gunn pupil and what is associated with

A
  • shining light into eye with optic neuritis causes both pupils to dilate instead of constrict
  • Multiple sclerosis
31
Q

how is Multiple sclerosis diagnosed

A
  • CNS lesion dissemination in TIME and SPACE
    • clinical findings + MRI findings
32
Q

CSF analysis of pt with Multiple sclerosis will likely show

A
  • presence of oligoclonal IgG bands
33
Q

evoked potential studies are used in Multiple sclerosis as a tool to

A
  • measure the time a stimulus (at eye, ear, or peripheral nerve) takes to reach cerebral cortex
34
Q

treatment of acute exacerbation of Multiple sclerosis

A
  1. glucocorticoids
    • large dose of oral prednisone x 3-7 d or IV methylprednisone
  2. Adrenocorticotropic injections if can’t use steroids
  3. plasma exchange if not responsive to steroids