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Neuromusculardisease
Neuromusculardisease
Dwayne McClerklin, MD
Assistant Professor
Department of Anesthesiology and Perioperative Medicine
MUSC
Myasthenia Gravis
Anesthetic Considerations
Preoperative continuation of AchEI
controversial (pros- potential decreased
postop weaknes; cons- altered action of
NMBAs, bowel hyperactivity)
Pts may be at increased risk of aspiration,
respiratory depression (go easy with
premedications)
Myasthenia Gravis
Anesthetic Considerations
Pts with ELS are sensitive to both
depolarizing and nondepolarizing NMBAs
Deep anesthesia with volatile agents is
usually sufficient for intubation
Muscular Dystrophies
Myotonic Dystrophy
Myotonia-slowing of relaxation after
muscle contraction in response to
electrical or percussive stimuli.
Myotonic Dystrophy (MD) is the most
common cause of myotonia
Muscular Dystrophies
Myotonic Dystrophy
S/Sx:Myotonia is the principal
manifestation early in the disease, but as
the disease progresses, muscle weakness
and atrophy become more prominent.
Other manifestations include
cardiomyopathy, gastric hypomotility, and
alveolar hypoventilation
Muscular Dystrophies
Myotonic Dystrophy
Anesthetic Considerations- Pts are
aspiration risks; avoid premedication due
to proneness to hypoventilation; avoid
Sux; Cis is a good choice; Neostigmine
can exacerbate myotonia; avoid
postoperative shivering
Myotonias
Myotonia Congenita
Path:caused by mutations of a gene on
chromosome 7q35 encoding a chloride
channel of the skeletal muscle fiber
surface membrane.
S/Sx:. The disease is confined to skeletal
muscle and produces no, minimal, or
nonprogressive weakness. There is no
cardiac involvement
Myotonias
Paramyotonia Congenita
S/Sx:Symptoms of paramyotonia congenita
include transient stiffness (myotonia) and,
occasionally, weakness after exposure to
cold temperatures;The stiffness worsens
with activity, in contrast to true myotonia,
thus the term "paramyotonia."
Myotonias
Anesthetic Considerations
Pts may demonstrate anabnormal response to
Sux and troublesome intraoperative myotonic
contractions (not associated with MH)
Avoid hypothermia.
NMBAs may paradoxically cause generalized
muscle spasms, including trismus, leading to
difficulty with intubation and ventilation
Periodic Paralysis
Anesthetic Considerations
Anesthetic management is directed toward
preventing attacks
Check K+ often
Glucose-containing intravenous fluids should
not be used in patients with hypokalemic
paralysis, whereas such solutions may benefit
patients with hyperkalemic and normokalemic
paralysis
Periodic Paralysis
Anesthetic Considerations
In patients with periodic paralysis, the
response to NMBAs is unpredictable
Pts with hypokalemic variant are sensitive to
nondepolarizing NMBAs
Sux is contraindicated in the hyperkalemic
variant
Maintain normothermia