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Special Pop Exam 1 > 1: Neuromuscular & Rare (Part 2) > Flashcards

1: Neuromuscular & Rare (Part 2) Flashcards

32-60

1
Q

⭐️
MG
Clinical hallmark
&
First notable sign

A

Clinical hallmark: skeletal muscle weakness that is aggravated by repetitive muscle use and improves with rest

First notable sign:

  • “weakness of extra-ocular muscles”
  • diplopia common presenting complaint (cranial nerves)
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2
Q

MG
-muscles especially affected
-other muscles affected

A

esp: facial expression, muscles of talking, chewing and swallowing

Weakness of arms/legs, bulbar symptoms or weakness of muscles of respiration

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3
Q

MG
Environmental, physical, and emotional factors can affect the disease such as….

A

surgery
pregnancy

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4
Q

MG
Myocarditis can occur in patients with

A

thymomas

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5
Q

Diagnosing MG
Clinical tests of fatigability:

A
  1. maintaining upward gaze
  2. holding out an affected limb
  3. respiratory function test
  • Electrophysiologic (EMG or supramaximal stimulation of peripheral nerve at 2 Hz)
  • Pharmacologic (Edrophonium/Tensilon Test)
  • Immunologic (anti-AChR antibody titer)
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6
Q

Which medication is given to diagnose MG?

A

Edrophonium

Pharmacologic (Edrophonium/Tensilon Test)

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7
Q

MG
anti-AChR antibody titer
common results

A
  • 85% have anti-AChR antibodies
  • 5% have no detectable antibodies
  • 10% may have antibodies against MuSK or LRP4
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8
Q

75-90% MG patients have
(3)

A

thymoma
thymic hyperplasia
thymic atrophy

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9
Q

⭐️
most common anterior mediastinal mass occurring in adults

A

Thymus hyperplasia

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10
Q

MG:
implications of thymoma, thymic hyperplasia & thymic atrophy

A
  • airway shift or compression if severe
  • Higher incidence of heart disease
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11
Q

Myasthenic Crisis
characteristics & causes

A

Characterized by:

  • Progression to severe muscle weakness
  • Respiratory failure (Vent)
  • Bundle Branch Blocks & A-fib

Causes:

  • Poor control of MG, underdosing of meds
  • Emotional stress
  • Hyperthermia
  • Pulmonary infection
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12
Q

⭐️
_____ exacerbates symptoms of MG in 33%

A

Pregnancy

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13
Q

T/F
Some pregnant women experience remission or no change in their MG.

A

True

33% of cases experience exacerbation

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14
Q

OB Exacerbations of MG are most likely to occur during…

A

first trimester or
6-weeks post-partum

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15
Q

T/F
Give Magnesium to the parturient with MG.

A

False
Magnesium should be avoided (for tocolysis and preeclampsia)?

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16
Q

⭐️
T/F
Anti-AChR antibodies cross the placenta.

A

True

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17
Q

The newborn of a mother with myasthenia gravis can suffer from a condition known as…

A

transient neonatal myasthenia

difficulty feeding, ptosis, facial weakness, & respiratory distress at birth

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18
Q

T/F
Transient Neonatal Myasthenia occurs in newborns of women with active MG

A

True

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19
Q

Transient Neonatal Myasthenia
Can begin …. & last ….

A

12-48 hours after birth

for weeks

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20
Q

Transient Neonatal Myasthenia
Spontaneous remission

A

usually at 2-4 wks when maternal antibodies clear circulation

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21
Q

Edrophonium testing (Tensilon Test)

A

improvement in strength = positive result

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22
Q

Which is best for the MS patient?
A) Succinylcholine
B) Remifentanil + Propofol Drip
C) Rocuronium

A

B) Remifentanil + Propofol Drip

avoid Suxx bc hyperK
avoid NDNMB if possible

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23
Q

Transient Neonatal Myasthenia
Testing & Treatment

A
  • Edrophonium testing (Tensilon Test)
  • Serologic testing
  • Treatment of symptom management and immunomodulation
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24
Q

conditions a/w MG

A
  • Thymus hyperplasia/thymoma
  • Hyper/hypothyroid
  • SLE
  • Rheumatoid arthritis
  • Ulcerative colitis
  • Pernicious anemia
  • Diabetes Mellitus
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25
Q

MG
“bulbar symptoms”

A

weakness of muscles innervated by the cranial nerves V, VII, IX, X, XI and XII

  • dysphagia
  • Difficulty chewing, choking on fluids, nasal regurgitation
  • Slurring of speech, dysphonia, dysarthria, dysphasia
  • Difficulty breathing
  • Weakness of neck muscles
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26
Q

Osserman Staging System for MG

“Im not going to hold you responsible for this”

A

fulminant: sudden, severe, and explosive event or process that can lead to lethality

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27
Q

⭐️
Treatment of MG:
Acetylcholinesterase Inhibitors (AchEI)

A
  • PO pyridostigmine, less muscarinic SEs than Neostigmine
  • Onset: 15-30 minutes
  • Peaks: 1-2 hours
  • Duration: 3-4 hours (60 mg can last 3-6 hours)
  • Daily dosage: 30-120 mg orally in divided doses
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28
Q

Acetylcholinesterase Inhibitors (AchEI) improve muscle strength for ______ but does not affect the course of the disease

A

several hours

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29
Q

Acetylcholinesterase Inhibitors (AchEI) increase concentration of Ach at the ____ membrane

A

postsynaptic

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30
Q

T/F
PO pyridostigmine has more muscarinic side effects than Neostigmine

A

False

Neostigmine has more muscarininc SEs

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31
Q

Cholinergic Crisis

A

ANTIcholinergic crisis: red, hot, dry, blind, mad

32
Q

Other MG Treatments bedsides Acetylcholinesterase Inhibitors (AchEI)
(2)

A

Immunosuppressants
&
Thymectomy

33
Q

Immunosuppressants for MG

A
  • Corticosteroids (reduces AChR antibody levels)
  • Cyclosporine
  • Azathioprine (interferes with the production of AChR antibodies)

Plasma exchange if respiratory compromise
IV immune globulin

34
Q

Thymectomy for MG

A
  • Indicated in patients with thymoma
  • Thymus gland hyperplasia
  • Drug-resistant MG
  • Transsternal (open) or Video-assisted transcervical
35
Q

In MG, the goal of a Thymectomy is…

A

remission or reduction in dosage of medications

36
Q

T/F
Thymoectomy is the only cure for MG.

A

False

Thymectomy Goal is remission or reduction in dosage of medications

37
Q

⭐️
Thymectomy For Myasthenia Gravis
Anesthetic Implications

A
  • Avoid muscle relaxants (TIVA/Inhalational and/or regional)
  • Discontinue or taper pyridostigmine dose before surgery
  • Sensitive to sedative medications, aspiration risk (Reglan)
38
Q

⭐️
Thymectomy For Myasthenia Gravis
Use of Paralytics

A

Anectine:

  • resistance or unpredictable (if not taking MG medications)
  • normal/prolonged response (taking MG meds)

NDMR: dramatic sensitivity (decrease in functional receptors) , especially when combined with inhalationals

Avoid muscle relaxants (TIVA/Inhalational and/or regional)

39
Q

Thymectomy For Myasthenia Gravis
Preop Assessment

A
  • medication list (steroids, pyridostigmine dose),
  • pulmonary function tests
  • ability to maintain airway ⭐️
  • CT/MRI of thymoma
  • EKG (bradycardia, rhythm issues)
40
Q

T/F
Thymectomy For Myasthenia Gravis
You know your pt has a dramatic sensitivity to succinylcholine.

A

False
Non-DNMB

Anectine:

  • resistance or unpredictable (if not taking MG medications)
  • normal/prolonged response (taking MG meds)

NDMR: dramatic sensitivity (decrease in functional receptors) , especially when combined with inhalationals

41
Q

Why are MG pts sensitive to Non-depolarizing NMB?

A

decreased number of postsynaptic receptors at the motor end plate

42
Q

⭐️
MG Patients should be informed that postoperative ____ may be needed

A

ventilation

43
Q

MG
Extubation

A
  • Reversal: cholinergic crisis, better to use Sugammadex?
  • Check NIF (> 30 cm H2O), head lift, cough, gag reflex, ensure full return of twitch
  • Qualitative vs. Quantitative twitch monitor
44
Q

T/F
When planning to extubate an MG pt, NIF should be <30 cm H2O

A

False
>30

head lift, cough, gag reflex, ensure full return of twitch, Quantitative twitch monitor

45
Q

T/F
Use of nonopioid analgesia is recommended for MG patients.

A

True

46
Q

Factors which Anticipate Postoperative Mechanical Ventilation

A
47
Q

Pyridostigmine daily dose of ….. should lead us to anticipate Postoperative Mechanical Ventilation

A

750 mg or more

48
Q

MG Thymectomy
Postop Care

A
  • Non-opioid pain meds
  • Pulmonary toilet
  • Avoid meds that depress respiration or delay extubation
  • Watch for postop respiratory failure
  • Cholinergic crisis vs Myasthenic crisis (edrophonium 10 mg)
49
Q

edrophonium differentiates ____ from ____

A

Cholinergic crisis vs Myasthenic crisis

10 mg

50
Q

T/F
Pyridostigmine does not cross the BBB.

A

True

physotigmine crosses BBB

51
Q

Lambert-Eaton Myasthenic syndrome is a/w…

A

small cell lung cancer

52
Q

In contrast to myasthenia gravis, ___ might improve the muscle weakness-related symptoms of Lambert-Eaton Myasthenic syndrome.

A

exercise

53
Q

Lambert-Eaton Myasthenic Syndrome (LEMS)

A
  • Autoimmune disorder, paraneoplastic (immune mediated associated with small cell lung cancer)
  • IgG antibodies are produced and attack pre-synaptic calcium channels
  • Decreased release of Ach from presynaptic terminals and decreased postjunctional response (muscle weakness)
54
Q

Lambert-Eaton Myasthenic Syndrome (LEMS)
pathophysiology r/t ACh

A
  • Decreased presynaptic release
  • Decreased postjunctional response (muscle weakness)
55
Q

T/F
In Lambert-Eaton Myasthenic Syndrome (LEMS), the number and quality of postjunctional AChRs is decreased.

A

False!
Number and quality of postjunctional AChRs is unaltered

Decreased presynaptic ACh release & postjunctional response (muscle weakness)

56
Q

⭐️
What does & does NOT improve Lambert-Eaton Myasthenic Syndrome (LEMS)?

A

Improvement with exercise
no improvement with anticholinesterase or steroids

57
Q

MG vs LEMS
post-junctional AChRs

A

MG: less
LEMS: # and quality unchanged

58
Q

LEMS
common population & complaint

A

Typically 50-70 year-old male
complains of proximal extremity weakness (hip and shoulder) that affects gait

59
Q

T/F
LEMS pts are sensitive to both depolarizing and nondepolarizers

A

True
avoid if possible

60
Q

LEMS
symptoms

A
  • Hip and shoulder weakness, difficulty walking and standing
  • Autonomic dysfunction
  • dry mouth & reduced sweating
  • erectile dysfunction
  • orthostatic hypotension
61
Q

Suspect LEMS in patients presenting with…

A

tumor/cancer and weakness

62
Q

These can help with LEMS symptoms

A

Prednisone and azathioprine

63
Q

Lambert-Eaton Myasthenic Syndrome
Treatment with surgery for cancer or/and

A

3,4-Diaminoyridine

64
Q

MG vs LEMS

A
65
Q
A
66
Q

Muscular Dystrophies (MD)

A

Hereditary disorders characterized by muscle fiber necrosis and regeneration, leading to muscle degeneration and progressive weakness

Muscular Dystrophies:

  • Duchenne’s
  • Becker’s
  • Myotonic
  • Limb-girdle
  • Congenital muscular dystrophy

Congenital Myopathies:

  • Central core disease
  • Centronuclear myopathy
67
Q

Muscular Dystrophies:

A
  • Duchenne’s
  • Becker’s
  • Myotonic
  • Limb-girdle
  • Congenital muscular dystrophy
68
Q

most common and severe type of Muscular Dystrophy

A

Duchenne’s

69
Q

Odds of Duchenne’s vs Becker’s

A

Duchenne’s: 1 per 3500 live male births

Becker’s (rare): 1 per 18,000

70
Q

Congenital Myopathies

A
  • Central core disease
  • Centronuclear myopathy
71
Q

Distribution of predominant muscle weakness in different types of muscular dystrophy

A

Distribution of predominant muscle weakness in different types of muscular dystrophy.

A:Duchenne-type and Becker type.
B:Emery–Dreifuss.
C: Limb-girdle.
D: Facioscapulohumeral.
E:Distal.
F: Oculopharyngeal.

72
Q

Duchenne’s Muscular Dystrophy (DMD)
Patho

A

Inherited, X-linked recessive disease, the muscles (incl myocardium) are gradually replaced with:
fat & connective tissue

Loss of functional dystrophin

73
Q

⭐️
Dystrophin

A

protein

plays a major role in:

  • stabilization of the muscle membrane
  • signaling between cytoskeleton and extracellular matrix
74
Q

Duchenne’s Muscular Dystrophy (DMD)
S/S

A

Proximal muscle weakness and gait issues, gradual onset of muscle wasting, contractures (kyphoscoliosis)

75
Q

Duchenne’s Muscular Dystrophy (DMD)
presentation & onset

A
  • childhood; 3-5 Y
  • proximal muscle weakness & painless atrophy in boys
76
Q

Duschenne Muscular Dystrophy
Manifestations across the lifespan

A

Special Pop Exam 1 (16 decks)

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