Musculoskeletal Dz Flashcards
Myasthenia Gravis Patho
- autoimmune dz
- IgG antibodies destroy POST-junctional receptors (nicotinic Ach)
- thymus gland plays key role; thymectomy relieves s/s
- Ach quantity sufficient
- not enough Ach receptors
- manifests as skeletal muscle wknss
Myasthenia gravis s/s
- key features:
~ muscle wknss later in day or w/ exercise
~ improves w/ rest - diplopia, ptosis (early)
- bulbar wknss (dysphagia, dysarthria, drooling)
- dyspnea w/ exertion
- proximal muscle wknss
- may require postop ventilation
Myasthenia Gravis is exacerbated by:
Apple - aminoglycosides
P - pregnancy
I - infection
E - electrolyte abnormalities
S - stress
How does myasthenia gravis affect pregnant mother & fetus?
- Pregnancy intensifies s/s in 1/3 of pregnant women
- Anti-Ach IgG antibodies cross placenta in 15-20% of neonates & cause wknss (can last up to 2-4 weeks)
- neonates require airway mgmt
Myasthenia Gravis Tx
- 1st line = oral pyridostigmine
- corticosteroids, cyclosporine, azathioprine, mycophenolate (immunosuppression)
- thymectomy (reduces Anti-AchR IgG)
- plasmapheresis
How can you differentiate b/t cholinergic crisis & myasthenic crisis?
- pyridostigmine OD –> cholinergic crisis
- Tensilon test = 1-2 mg IV edrophonium
~ muscle wknss worse = cholinergic crisis –>
tx w/ anticholinergic
~ muscle wknss better = myasthenic crisis –>
tx w/ anticholinesterase,
immunosuppression, plasmapheresis
How do patients w/ myasthenia gravis respond to sux?
- Fewer nicotinic Ach (Nm) receptors
- Resistant
- Require increased dose (1.5- 2.0 mg/kg)
- pyridostigmine prolongs DOA of sux
How do patients w/ myasthenia gravis respond to ND-NMB?
- Fewer nicotinic Ach (Nm) receptors
- Sensitive (potency increased)
- Reduce dose by 1/2 to 2/3
5 perioperative risks that increase postop mechanical ventilation in pt w/ myasthenia gravis
- Dz > 6 years
- pyridostigmine > 750 mg/day
- VC < 2.9 L
- COPD
- Surgical approach: median sternotomy> transcervical thymectomy
Eaton-Lambert Syndrome (Myasthenic Syndrome) Patho
- IgG-mediated destruction of PRE-synaptic VG
Ca+ channels - Ca+ entry in presynaptic neuron decreased
- Decreased amount of Ach released into
synaptic cleft - 60% have small-cell lung CA (oat-cell)
ELS S/S
- Proximal muscles affected
- wknss worse in AM, gets better throughout
day - Respiratory muscles & diaphgram weak
- ANS dysfunction:
~ orthostatic hypotension
~ slowed gastric motility
~ urinary retention
ELS Tx
- 3, 4- diaminopyridine (DAP)
~ increases Ach release from presynaptic
nerve
AchE not helpful
Tensilon test does NOT aid diagnosis
How do pt w/ ELS respond to NMBs?
- Sensitive to sux & NDNMBs
- Reduce dose
ELS Mgmt
- Reversal may be inadequate
- High risk postop resp failure
ELS vs. Myasthenia Gravis
Guillain-Barre (acute idiopathic polyneuritis)
- Destruction of myelin prevents nerve conduction in peripheral nerves
- Precipitated by flu-like illness followed by paralysis
- Causes: epstein-barr, campylobacter, cytomegalovirus
Guillain-Barre S/S
- Flaccid paralysis begins in distal extremtities & ascends bilaterally
- Impaired ventilation (intercostal muscles)
- Difficulty swallowing (facial & pharyngeal muscles)
- Paresthesias, numbness, pain
- ANS dysfunction
~ tachycardia, bradycardia, hyper/hypotension, diaphoresis, anhidrosis
Guillain-Barre Tx
- plasmapheresis
- IV IgG
Guillain-Barre Mgmt
- avoid sux (hyperkalemia)
- sensitive to NDNMBs (decrease dose)
- increased aspiration risk
- may require postop ventilation
- autonomic dysfx = HD instability = a-line
- exaggerated response to indirect-acting sympathomimetics (avoid ephedrine)
Hypokalemic periodic paralysis
- Skeletal muscle wknss follows a glucose-insulin infusion
- pt becomes weak after K+ decreases
- Ca+ channelopathy
AVOID:
- glucose solutions
- K+ wasting diuretics
- B2 agonists
- Sux
- hypothermia
Safe:
- NDNMBs
- Acetazolamide
Tx: Acetazolamide (creates non-anion gap acidosis, protects against hypokalemia)
Hyperkalemic periodic paralysis
- skeletal muscle wknss follows oral K+ administration
- pt becomes weak after K+ increases
- Na+ channelopathy
AVOID:
- Sux
- LR (solutions w/ K+)
- hypothermia
Safe: everything listed in AVOID on hypokalemia card + acetazolamide
Tx: Acetazolamide (facilitates K+ excretion)
MH patho
- Inherited disease of skeletal muscle characterized by disordered Ca+ homeostasis
- Genetic mutation of ryanodine receptor (RYR1)
- Defective RYR1instructs SR to release too much Ca+ into cell
- Increase O2 consumption
- Increased CO2 production
Consequences of increased intracellular Ca+
- rigidity from sustained contraction
- accelerated metabolic rate
- depletion of ATP
- increased O2 consumption
- increased CO2 production
- increased heat production
- mixed resp & lactic acidosis
- sarcolemma breaks down
- K+ & myoglobin leak out into blood
Conditions NOT associated w/ MH
- Becker MD
- Neuroleptic malignant syndrome
- myotonia congenita
- myotonic dystrophy
- osteogenesis imperfecta
3 conditions associated w/ MH
- King-Denborough syndrome
- Central core dz
- Multiminicore dz
MH triggers
- VA
- sux
MOST sensitive indicator of MH
EtCO2
Early s/s MH
- tachycardia
- tachypnea
- masseter spasm
- warm soda lime
- irregular heart rhythm