Anesthesia of patients with motor neuron disease

1. Anesthesia of patients with motor neuron disease Presentation by: SC 廖伯峰 江毅彥

2. 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

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

4. 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 (-)

5. Spirometry(2001) • Normal standard spirometry • Normal diffusion capacity

6. Spirometry(2004) * Normal screening spirometry

9. 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

10. About motor neuron disease • Lower motor neuron (LMN): axons synapse directly on skeletal muscles • Upper motor neuron (UMN): motor cortex →pyramidal tract →LMN

11. LMN loss signs: Flaccid paralysis Fasciculations Hypotonia Hyporeflexia, areflexia UMN loss signs: Initially weak and flaccid Eventually spastic Hypertonia Hyperreflexia Babinski sign

13. Amyotrophic lateral sclerosis • Amyotrophy: biopsy →muscle atrophy • Lateral sclerosis: lateral column. gliosis →firmness • Lou Gehrig's disease

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

15. epidemiology • 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

16. 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

18. 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

19. Respiratory system • Breathlessness • Nocturnal hypoventilation→ morning headache, daytime hypersomnolence • Poor cough • Recurrent pulmonary aspiration • Recurrent pneumonia • Respiratory failure • Die

21. Pathogenesis • Not well defined • SOD1 mutation →free radical accumulations • Glutamate (EAAT2, astroglial cell) • VEGF↓

23. Discussion Anesthesia in patients with ALS

24. Miller's anesthesia ,2005 Anesthesia and neurosurgery, 2001 • Responses to muscle relaxants • Bulbar dysfunction • Lung function: spirometry • Regional anesthesia VS general anesthesia

25. 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

26. 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

27. 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

28. 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

29. 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

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

31. 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

32. 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 Pre-OP management

33. 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

34. 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

35. Case report Ⅰ • 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

36. 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

37. Case report Ⅱ • 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.

38. 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

39. 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

40. 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

41. 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

42. 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

43. 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?

44. 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