Anesthesia of patients with motor neuron disease

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Anesthesia of patients with motor neuron disease

• Presentation by SC ???
• ???

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History

• 53 y/o Female
• Alcohol(), Smoking () 1/2 PPD
• General appearance weakness
• Vital sign T/P/R36.2/84/18, BP120/60 mmHg,
• Chest Symmetric expansion.
• Extremities Movable but weakness

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History

• 1.Breast tumor r/o ductal adenocarcinoma
• 2. Amyotrophic lateral sclerosis(2001)
• 3. Gastric ulcer
• 4. Alcoholic with chronic pancreatitis

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• CN I not performed
• CN II visual field intact visual acuity not
  performed
• CN III, IV VI EOM full
• CN V facial sensation intact
• CN VII facial palsy (-)
• CN VIII intact
• CN IX X gag reflex ( / )
• CN XI decreased muscle power of SCM and
  trapezious muscle
• CN XII tongue deviation (-), no atrophy or
  fasciculation
• Reflex Babinskis sign (-), Hoffman sign (-)

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Spirometry(2001)
Observed Predicted predicted
FVC(L) 2.96 2.77 106.7
FEV1.0(L) 2.60 2.37 109.7
FEV1.0 () 87.8 85.2
VC(L) 2.83 2.77 102.0
RV(L) 1.68 1.73 97.4

• Normal standard spirometry
• Normal diffusion capacity

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Spirometry(2004)
Observed Predicted predicted
FVC(L) 2.78 2.64 105.3
FEV1.0(L) 2.19 2.23 98.2
FEV1.0 () 78.8 84.4
Normal screening spirometry
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About motor neuron disease

• Selectivity of neuronal cell deathmotor neuron
  only
• (except 1.ocular motility 2.parasympathetic
  neuron in sacral spinal cord)
• Light microscopy sensory, coordination of
  movement, cognitive process remain intact
• Immunostaining ubiquitin also in nonmotor
  systemsmarker for degeneration
• Glucose metabolism

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About motor neuron disease

• Lower motor neuron (LMN) axons synapse directly
  on skeletal muscles
• Upper motor neuron (UMN) motor cortex ?pyramidal
  tract ?LMN

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• LMN loss signs
• Flaccid paralysis
• Fasciculations
• Hypotonia
• Hyporeflexia, areflexia

• UMN loss signs
• Initially weak and flaccid
• Eventually spastic
• Hypertonia
• Hyperreflexia
• Babinski sign

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Amyotrophic lateral sclerosis

• Amyotrophy
• biopsy ?muscle atrophy
• Lateral sclerosis
• lateral column.
• gliosis ?firmness
• Lou Gehrig's disease

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loss of anterior horn cells, the anterior
(ventral) spinal motor nerve roots demonstrate
atrophy
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epidemiology

• Prevelence3 to 5 per 100,000
• Western pacific
• Sporadic (heavy metal?)
• Inherited( AD) 5 to 10
• Males
• 40 to 70 y/o (55)
• Median survival 3 to 5 years

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Amyotrophic lateral sclerosis

• Upper and lower motor neuron
• Progressive weakness
• Exclusion of alternative diagnosis
• Bulbar, cervical, thoracic, lumbosacral motor
  neurons
• 1 ?possible
• 2 ?probable
• 3 ?definite

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Clinical manifestation

• Asymmetric weakness, usually one of distally limb
  first
• Cramping with volitional movements, typically in
  the morning
• Wasting and atrophy of muscle
• Fasciculation
• Hyperactivity of muscle stretch reflexes
• Difficulty of chewing, swallowing, dysarthria,
  exaggeration of emotion expressions
• Respiratory system

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Respiratory system

• Breathlessness
• Nocturnal hypoventilation? morning headache,
  daytime hypersomnolence
• Poor cough
• Recurrent pulmonary aspiration
• Recurrent pneumonia
• Respiratory failure
• Die

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Pathogenesis

• Not well defined
• SOD1 mutation ?free radical accumulations
• Glutamate (EAAT2, astroglial cell)
• VEGF?

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Discussion

• Anesthesia in patients with ALS

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Miller's anesthesia ,2005 Anesthesia and
neurosurgery, 2001

• Responses to muscle relaxants
• Bulbar dysfunction
• Lung function spirometry
• Regional anesthesia VS general anesthesia

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Responses to muscle relaxants

• Predisposed to SCC-induced hyperkalemia because
  of denervation and atrophy of skeletal muscles
• Patient with motor neuron disease are at risk for
  hyperkalemia when using SCC because of
  upregulation of nAChRs
• SCC is best avoided

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• ALS patient, in addition, presynaptic impairment
  of neuromuscular transmission, explains their
  hypersensitivity to nondepolarizing neuromuscular
  blockers
• Increased sensitivity to nondepolarizing muscle
  relaxants ? either relaxants be avoided
  altogether or shorter-acting relaxants be used

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Bulbar dysfunction

• In late stage of the disease, reduced respiratory
  muscle reserve and abnormal airway protective
  reflexes ? increased risk for respiratory
  depression and aspiration secondary to the use of
  sedative and anesthetic drugs
• Aspiration prophylaxis should be considered no
  evidence
• Placement of a feeding tube

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Lung function

• Respiratory muscle weakness frequently develops
• Pre-OP ventilatory impairment may help predict
  anesthetic risk ? one small study 40 FVC/FEV1
• Ventilatory support in the immediate post-OP
  period may be necessary

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Regional VS general anesthesia

• Epidural anesthesia used in ALS patients without
  reported untoward effect
• Regional anesthesia is preferable to GA
• RA may facilitate progression of
  neurodegenerative disease evidence is anecdotal

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• There is evidence of sympathetic hyperactivity
  and autonomic failure accompanied by reduced
  baroreflex sensitivity

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Handbook For Anesthesia And Co-Existing Disease,
2002

• Pre-OP assessment
• History and Examination general details, bulbar
  function, respiratory function
• Investigations to confirm diagnosis, routine
  works, chest radiography, LFT,ABG, tests of
  diaphragmatic function, videofluoroscopy

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Pre-OP management

• Premedication
• Opioid should be avoided
• Small dose of benzodiazepine
• Prophylaxis against pulmonary aspiration (i.e. an
  H2-receptor antagonist)

• Monitoring
• ECG
• BP
• SpO2
• EtCO2
• Neuromuscular function monitoring

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Induction and maintenance of anesthesia

• Tracheal extubation should be performed with the
  patient fully awake to ensure maximal function of
  the laryngeal reflexes
• Regional anesthesia if appropriate, better than
  GA, level of block not to compromise an already
  weak respiratory musculature

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Post-OP management

• Effective post-OP pain relief without the use of
  agents that depress respiratory
• Post-OP ventilation may be required and weaning
  may be prolonged

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Case report ?

• 46 y/o woman with emergency operation for ileus
• 65 y/o woman with emergency operation for gastric
  fistula malfunction
• 63 y/o man scheduled for a surgery of rectal
  cancer
• 49 y/o man scheduled for gastrectomy

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• Spinal or/and epidural anesthesia without muscle
  relaxants
• IV propofol(3mg/kg) and sevoflurane (5)
  inhalation of 2-3 sevoflurane and single IV
  vecuronium 1 mg
• GA with sevoflurane and epidural anesthesia with
  lidocaine vecuronium

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Case report ?

• Patient undergoing abdominal hysterectomy
• Epidural anesthesia with 2 lidocaine
  continuous infusion of low dose propofol for
  sedation
• Epidural morphine provides excellent post-OP pain
  relief without respiratory complication

Chen LK, Chang Y, Liu CC, Hou WY.Department of
Anesthesiology, National Taiwan University
Hospital, Taipei, R.O.C.
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Case report ?

• General anaesthesia in a patient with motor
  neuron disease
• 2004 European Academy of anaesthesiology
• 56 y/o man with pancreatic carcinoma scheduled
  for elective Whipples surgery

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• 5-h operation ? 8-h after, patient recovered
  consciousness and ventilated with CPAP ? 5-h
  after, extubated with normal breathing pattern ?
  3-h after, secondary surgery ? 3-h operation ?
  3-h after
• Without SCC several complications
• Low-dose cisatracurium may be a good choice

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• For major surgery, using low-dose of
  cisatracurium and propofol is possible
• Repeated surgery may lead to a considerable
  increase in respiratory complications
• Spirometry should be performed and the use of
  neuromuscular monitoring when planning GA

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Case report ?

• Use of rapacuronium in a child with spinal
  muscular atrophy
• Paediatric anaesthesia 2001
• 18 month-old girl with SMA diagnosed at 6 m/o
• Scheduled for placement of a percutaneous
  jejunostomy tube

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• Thiopental sodium, alfentanil, lidocaine for
  induction, and mask ventilation with 2
  isoflurane
• Rapacuronium 9 mg (1 mg/kg) for emergency airway
  control
• Within 10 min, partial recovery of the diaphragm
  was observed ? 20 min after, TOF responses ? 75
  min after, operation done
• Reversal with neostigmine 0.6 mg and
  glycopyrrolate 0.12 mg IV

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• Guidelines are entirely based on experience with
  the adult form of anterior horn cell degeneration
  (ALS)
• Induction with propofol in combination with
  sevoflurane might have avoided laryngospasm and
  the use of muscle relaxants
• A lesser dose might have been sufficient to break
  laryngospasm
• TOF a good monitor?

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Conclusion

• SCC is best avoided, reported to cause
  rhabdomyolysis and hyperkalemia from denervated
  muscles
• Use of neuromuscular monitoring when planning GA
  TOF ?
• Muscle relaxants should be avoided altogether or
  shorter-acting/low-dose muscle relaxants be used