1、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: Symmetric expansion. Extremities: Movable but weakness,History,1.Breast tumor r/o ductal
2、 adenocarcinoma 2. Amyotrophic lateral sclerosis(2001) 3. Gastric ulcer 4. Alcoholic with chronic pancreatitis,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 r
3、eflex ( + /+ ) CN XI: decreased muscle power of SCM and trapezious muscle CN XII: tongue deviation (-), no atrophy or fasciculation Reflex: Babinskis sign (-), Hoffman sign (-),Spirometry(2001),Normal standard spirometry Normal diffusion capacity,Spirometry(2004),* Normal screening spirometry,About
4、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 degenera
5、tion Glucose metabolism,About motor neuron disease,Lower motor neuron (LMN): axons synapse directly on skeletal muscles Upper motor neuron (UMN): motor cortex pyramidal tract LMN,LMN loss signs: Flaccid paralysis Fasciculations Hypotonia Hyporeflexia, areflexia,UMN loss signs: Initially weak and fla
6、ccid Eventually spastic Hypertonia Hyperreflexia Babinski sign,Amyotrophic lateral sclerosis,Amyotrophy:biopsy muscle atrophy Lateral sclerosis: lateral column. gliosis firmness Lou Gehrigs disease,loss of anterior horn cells, the anterior (ventral) spinal motor nerve roots demonstrate atrophy,epide
7、miology,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,
8、lumbosacral motor neurons1 possible 2 probable3 definite,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 chew
9、ing, swallowing, dysarthria, exaggeration of emotion expressions Respiratory system,Respiratory system,Breathlessness Nocturnal hypoventilation morning headache, daytime hypersomnolence Poor cough Recurrent pulmonary aspiration Recurrent pneumonia Respiratory failure Die,Pathogenesis,Not well define
10、d SOD1 mutation free radical accumulations Glutamate (EAAT2, astroglial cell) VEGF,Discussion,Anesthesia in patients with ALS,Millers anesthesia ,2005 Anesthesia and neurosurgery, 2001,Responses to muscle relaxants Bulbar dysfunction Lung function: spirometry Regional anesthesia VS general anesthesi
11、a,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,ALS patient, in addition, presynaptic imp
12、airment 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,In late stage of the disease, reduc
13、ed 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,Respiratory muscle w
14、eakness 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,Epidural anesthesia used in ALS patients without reported untoward effect Regio
15、nal anesthesia is preferable to GA RA may facilitate progression of neurodegenerative disease: evidence is anecdotal,There is evidence of sympathetic hyperactivity and autonomic failure accompanied by reduced baroreflex sensitivity,Handbook For Anesthesia And Co-Existing Disease, 2002,Pre-OP assessm
16、ent 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 Opioid: should be avoided Small dose of benzodiazepin
17、e Prophylaxis against pulmonary aspiration (i.e. an H2-receptor antagonist),Monitoring ECG BP SpO2 EtCO2 Neuromuscular function monitoring,Induction and maintenance of anesthesia,Tracheal extubation should be performed with the patient fully awake to ensure maximal function of the laryngeal reflexes
18、 Regional anesthesia: if appropriate, better than GA, level of block not to compromise an already weak respiratory musculature,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,Case rep
19、ort ,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,Spinal or/and epidural anesthesia without muscle relaxants IV propofol(3mg/kg) and sevof
20、lurane (5%) + inhalation of 2-3% sevoflurane and single IV vecuronium 1 mg GA with sevoflurane and epidural anesthesia with lidocaine + vecuronium,Case report ,Patient undergoing abdominal hysterectomy Epidural anesthesia with 2% lidocaine + continuous infusion of low dose propofol for sedation Epid
21、ural 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 ,General anaesthesia in a patient with motor neuron disease 2004 European Academy of a
22、naesthesiology 56 y/o man with pancreatic carcinoma scheduled for elective Whipples surgery,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 c
23、omplications Low-dose cisatracurium: may be a good choice,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
24、 GA,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,Thiopental sodium, alfentanil, lidocaine for induction, and mask ventilation with 2% isofl
25、urane 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,Guidelines are entirely based on experience with the ad
26、ult 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,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,
