Barash 24: Rare NM Flashcards

1
Q

muscular dystrophy
agents to avoid & why

A

Succinylcholine and/or halogenated anesthetics

massive release of intracellular contents resulting in hyperkalemic cardiac arrest.

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2
Q

Which NM condition can cause a cardiac conduction delay manifesting as third-degree atrioventricular block?

A

Myotonic dystrophy

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3
Q

T/F:
myasthenia gravis is exquisitely sensitive to depolarizing muscle relaxants

A

False
NON-depolarizing

use Short-acting muscle relaxants
objective monitoring of neuromuscular function

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4
Q

can produce myasthenic syndrome

A

Many types of cancer
small-cell carcinoma of the lung

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5
Q

T/F:
Patients with multiple sclerosis can have an exacerbation of their neurologic symptoms despite a well-managed anesthetic.

A

True

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6
Q

most sensitive sign of malignant hyperthermia

A

An unexpected increase in etCO2

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7
Q

constant risks to patients with glycogen storage diseases

A

Hypoglycemia and metabolic acidosis

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8
Q

mucopolysaccharidoses
airway management during anesthesia

A

deposition of mucopolysaccharides in the upper airway of patients with mucopolysaccharidoses often complicates

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9
Q

Repeated episodes of sickling in patients with sickle cell disease cause ____ that increases perioperative risks

A

pulmonary hypertension

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10
Q

Rheumatoid arthritis is a multisystem disease that causes

A

subclinical cardiac and pulmonary dysfunction

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11
Q

Esophageal dysfunction in these patients increases the risk of aspiration pneumonitis

A

scleroderma and dermatomyositis

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12
Q

epidermolysis bullosa

A

can have undiagnosed dilated cardiomyopathy

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13
Q

Table 24-1
Types of Muscular Dystrophy and Congenital Myopathies

A
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14
Q

Distribution of predominant muscle weakness in different types of muscular dystrophy

A

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

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15
Q

Table 24-3
Skeletal Muscle Channelopathies

A
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16
Q

Table 24-5
Clinical Presentations of Myasthenia Gravis

A
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17
Q

Table 24-6
Osserman Staging System for Myasthenia Gravis

A
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18
Q

Table 24-7
Comparison of Myasthenic Syndrome and Myasthenia Gravis

A
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19
Q

MS
its neurologic dysfunction + potent therapeutic drugs can manifest during the perioperative period as….

A

autonomic dysfunction, myopathy, cardiotoxicity,
and sensitivity or resistance to paralytics

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20
Q

Can we give Suxx to MS?

A

could produce an exaggerated release of potassium

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21
Q

T/F:
postoperative respiratory support is likely needed for MS

A

True
Respiratory muscle weakness and poor respiratory control

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22
Q

levodopa and anesthesia

A

half-life of levodopa is short!
stopping for 6 to 12+ hours can result in severe skeletal muscle rigidity that interferes with ventilation

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23
Q

Parkinsons and anesthesia

A
  • autonomic dysfunction is common
  • orthostatic hypotension that may be aggravated by the vasodilatory effects of anti-Parkinson drugs and inhaled anesthetics.
  • Excessive salivation and esophageal dysfunction(aspiration pneumonitis)
  • Upper airway obstruction (try anti-Parkinson agents) postoperative confusion and hallucinations
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24
Q

preferred anesthesia method for Parkinsons

A

Awake techniques with sedation and local anesthesia
so intraoperative testing of the stimulator can be performed

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25
Q

The major cause of dementia in the United States

A

alzhemiers

26
Q

T/F:
Alzheimers characterstics include seizures

A

True

loss of cognition, poor decision making, language deterioration,
gait disturbances,
seizures, agitation, and psychosis

27
Q

T/F:
Patients and their families should be advised that POCD may occur in alzheimers patients

postoperative cognitive dysfunction

A

True

28
Q

T/F:
Sedative premedication may worsen mental confusion in Alzheimers.

A

True
use w caution

29
Q

Which anticholinergic is ok for alzheimers?

A

glycopyrrolate (does not cross BBB)

rather than atropine or scopolamine

30
Q

Alzheimers patients receiving cholinesterase inhibitors may have a prolonged response to ______

A

succinylcholine

31
Q

Huntington Disease
what to use
what not to

A
  • recovery from propofol may be faster
  • Short-acting relaxants are preferable
  • Decreased plasma cholinesterase activity may prolong the response to succinylcholine
  • Spinals are ok
32
Q

Airway of the Huntington Disease patient

A

pharyngeal muscles become dysfunctional
risk of aspiration pneumonitis increases

Delayed emergence & increased likelihood of respiratory complications

33
Q

Amyotrophic Lateral Sclerosis
and paralytics

A

may be very sensitive to nondepolarizing muscle relaxants.
Avoid Suxx (massive release of potassium)

postoperative ventilatory support is likely

34
Q

Malignant Hyperthermia

A

pharmacogenetic disorder of skeletal muscle
hypermetabolic process associated with significant morbidity and mortality

35
Q

MH triggers

A

succinylcholine or halogenated, inhaled anesthetics.
extreme physiologic stress or heat exhaustion

36
Q

MH
what gene is affected and what happens

A

mutation of the ryanodine receptor
uncontrolled release of calcium (Ca+2) from the sarcoplasmic reticulum (SR)

  • sustained muscle contraction/rigidity
  • metabolic and respiratory acidosis
  • hypercarbia
  • tachycardia, hyperthermia
  • rhabdomyolysis, and hemodynamic instability.
37
Q

Nonspecific signs of MH

A

tachycardia, tachypnea (spontaneous ventilation), and hypertension.

Muscle rigidity, masseter spasm, and respiratory and metabolic acidosis appear afterward

38
Q

T/F:
Hyperthermia may occur early OR late in an MH episode

A

True

39
Q

Conditions that may mimic MH

A

sepsis, hyperthyroidism, and NMS

40
Q

most definitive treatment for MH

A

dantrolene, a hydantoin derivative that inhibits the pathologic release of Ca+2

41
Q

dose of dantrolene

A

initial: 2.5 mg/kg
repeated until reaction has subsided
may require 10 to 20 mg/kg of dantrolene

42
Q

Other than Dantrolene/Ryanodex, what do we do?

A
  • STOP halogenated, inhaled anesthetic
  • hyperventilate with 100% O2
  • stop surgery under IV anesthesia
  • Charcoal filters to purge the system
  • treat acidosis and hyperkalemia
  • active cooling
  • maintain urinary output
43
Q

Titrate dantrolene to …

A

heart rate, temperature, and PaCO2

44
Q

Cardiac arrhytmias in MH
what to give
what NOT to give

A

give lidocaine

DO not give CCB

45
Q

MH
Correction of hyperkalemia

A

Glucose, insulin, bicarbonate, hyperventilation
Calcium if hyperkalemia causes cardiac dysfunction

46
Q

most definitive test for MH susceptibility

A

caffeine–halothane contracture test

47
Q

T/F:
nitrous oxide is safe for MH patients or those susecptible to it.

A

True

48
Q

T/F:
Give Dantrolene prophylactically if pt has MH or susecptible to it.

A

FALSE

49
Q

Plasma cholinesterase (pseudocholinesterase, butyrylcholinesterase) is an enzyme synthesized in ….

A

the liver

49
Q

Table 24-11
Safe Versus Unsafe Drugs in Malignant Hyperthermia

A
50
Q

Cholinesterase Disorders
most significant complication for the anesthesiologist

A

prolonged apnea after succinylcholine

51
Q

Drugs that may interfere with succinylcholine metabolism

A

neostigmine
pyridostigmine
echothiophate
cyclophosphamide
chlorpromazine
organophosphate insecticides

52
Q

If pt has plasma cholinesterase abnormality, how should u manage paralysis?

A

be certain that recovery from the initial dose of succinylcholine has occurred before giving more muscle relaxant

Mechanical ventilation and adequate sedation should be continued until full recovery of neuromuscular function is assured.

53
Q

Table 24-14
Classic Cholinesterase Genotypes
&
Dibucaine #

A
54
Q

Osteogenesis Imperfecta
anesthesia do’s and don’ts

A
  • gently positioned b/c fractures.
  • assess upper airway & cervical ROM
  • Megalocephaly & short neck = hard to DL; alternative techniques
  • echo (valvular heart disease common)
  • Regional avoided (spinal trauma & platelet dysfunction)
55
Q

Which condition may result in elevated Temp but is not actually linked to MH?

A

Osteogenesis Imperfecta
Mild intraoperative temperature elevation but no evidence of increased risk for MH

56
Q

Table 24-24
Extra-Articular Manifestations of Rheumatoid Arthritis

A
57
Q

occurs in 20% to 50% of RA patients

A

Pericarditis

can produce restrictive pericarditis and cardiac tamponade

58
Q

RA
anesthesia

A
  • consider Awake intubation, video laryngoscopy, or flexible, fiberscope
  • may need smaller ETT (Cricoarytenoid arthritis = laryngeal edema)
  • bleed risk: Aspirin and other anti-inflammatory drugs
  • hold anti-TNF before surgery (infection risk)
59
Q

T/F:
Patients with SLE are at increased risk for infection

A

True

60
Q

Which SLE meds affect our anesthesia?

A

Cyclophosphamide inhibits cholinesterase and may prolong the response to succinylcholine

Azathioprine can increase dose requirements for nondepolarizing muscle relaxants.