Transcript Anesthesia of patients with motor neuron disease Presentation by: SC 廖伯峰 江毅彥 History       53 y/o Female Alcohol(+), Smoking (+) 1/2 PPD General appearance: weakness Vital sign: T/P/R=36.2/84/18, BP:120/60 mmHg, Chest:

Anesthesia of patients
with
motor neuron disease
Presentation by: SC 廖伯峰
江毅彥
History
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53 y/o Female
Alcohol(+), Smoking (+) 1/2 PPD
General appearance: weakness
Vital sign: T/P/R=36.2/84/18, BP:120/60
mmHg,
Chest: Symmetric expansion.
Extremities: Movable but weakness
History
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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: Babinski’s sign (-), Hoffman sign (-)
Spirometry(2001)
Observed
Predicted
%predicted
FVC(L)
FEV1.0(L)
%FEV1.0 (%)
VC(L)
2.96
2.60
87.8
2.83
2.77
2.37
85.2
2.77
106.7
109.7
RV(L)
1.68
1.73
97.4
•Normal standard spirometry
• Normal diffusion capacity
102.0
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
About motor neuron disease
Selectivity of neuronal cell death—motor
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
systems—marker for degeneration
 Glucose metabolism
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About motor neuron disease
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Lower motor neuron (LMN): axons synapse
directly on skeletal muscles
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Upper motor neuron (UMN): motor cortex
→pyramidal tract →LMN
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LMN loss signs:
1)
2)
3)
4)
Flaccid paralysis
Fasciculations
Hypotonia
Hyporeflexia,
areflexia
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UMN loss signs:
1) Initially weak and
flaccid
2) Eventually spastic
3) Hypertonia
4) Hyperreflexia
5) Babinski sign
Amyotrophic lateral sclerosis
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Amyotrophy:
biopsy →muscle atrophy
Lateral sclerosis:
lateral column.
gliosis →firmness
Lou Gehrig's disease
loss of anterior horn cells, the anterior (ventral) spinal motor
nerve roots demonstrate atrophy
epidemiology
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Prevelence:3 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
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
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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
Respiratory system
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Breathlessness
Nocturnal hypoventilation→ morning
headache, daytime hypersomnolence
Poor cough
Recurrent pulmonary aspiration
Recurrent pneumonia
Respiratory failure
Die
Pathogenesis
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Not well defined
SOD1 mutation →free radical accumulations
Glutamate (EAAT2, astroglial cell)
VEGF↓
Discussion
Anesthesia in patients with ALS
Miller's anesthesia ,2005 Anesthesia
and neurosurgery, 2001
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Responses to muscle relaxants
Bulbar dysfunction
Lung function: spirometry
Regional anesthesia VS general anesthesia
Responses to muscle relaxants
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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
Bulbar dysfunction
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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
Lung function
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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
Regional VS general anesthesia
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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
Handbook For Anesthesia And CoExisting 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
Pre-OP management
Premedication
Monitoring
 Opioid: should be
 ECG
avoided
 BP
 Small dose of
 SpO2
benzodiazepine
 EtCO2
 Prophylaxis against
 Neuromuscular function
pulmonary aspiration (i.e.
monitoring
an H2-receptor
antagonist)
Induction and maintenance of
anesthesia
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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
Post-OP management
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Effective post-OP pain relief without the use of
agents that depress respiratory
Post-OP ventilation may be required and
weaning may be prolonged
Case report Ⅰ
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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
Case report Ⅱ
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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.
Case report Ⅲ
General anaesthesia in a patient with motor
neuron disease
 2004 European Academy of anaesthesiology
 56 y/o man with pancreatic carcinoma
scheduled for elective Whipple’s 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
Case report Ⅳ
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?
Conclusion
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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