Guillain Barre’ syndrome(GBS) and Anesthesia consideration

Patients with GBS need special care when coming to the surgery. They have a high risk of aspiration, airway compromise, autonomic instability, altered response to NMBs. It is the duty of the anesthesia providers to recognize those problems and minimize the complications.

1. Guillain Barre’ syndrome(GBS) and Anesthesia consideration Dr. Tenzin Yoezer 15/6/2019 KGUMSB

2. History 52 yr old female, previously well with history of chronic alcohol abuse presented with bilateral lower limb weakness and pain for 3 days. Limb weakness was progressive and on 4 th day onwards became bed bound 1 week prior to limb weakness had self limiting loose stool While in P/ling hospital developed seizure(type of seizure not mentioned) Referred to JDWNRH for further mx While in ER developed two episodes of seizure and didn ’ t gain consciousness

3. History Was intubated and kept in AICU O/E – mild icteric Other examination were unremarkable except Limb examination: Both UL and LL power – grade 2 Tone – flaccid Reflex – not mentioned Initial lab reports at P/ling: WBC- 18 LFT - normal Neu – 86% RFT - normal Hb – 12 USG - normal Plt – 324 CXR - NAD

4. Managed as: Peripheral alcohol neuropathy Alcohol withdrawal seizure Sepsis Aspiration pneumonia GBS AB – Ceftriaxone, Gentamycin

5. All the vitals stable throughout the illness CT brain: atrophy with mild ventricular dilatation ECG - sinus rhythm ESR – 40 CSF studies – no cells, protein -137.8 mg/ dL Neurophysiological studies – report not available

6. On 14 th day of illness IVIg was initiated after consulting neurologist(HVO): for 5 days IVIg 400 mg IV drip 20 mL/ hr x 1 hr 40 mL/ hr x 1hr 60 mL/ hr x 1 hr 80 mL/ hr x 1 hr

7. While in the ward developed 4 th degree sacral bed sore. Brought in OT for W/D Face mask with TIVA: Inj Midazolam 2mg Inj Fentanyl 50 mcg Inj Propofol 100 + 20 mg

8. Final diagnosis Acute inflammatory demyelinating polyradiculopathy Complex partial seizure Grade IV seizure

9. Guillain Barre’ Syndrome

10. Introduction First described in 1859 Acute inflammatory demyelinating peripheral polyneuropathy usually secondary to immunologic response to viral or bacterial infection (usually respiratory or GI ) Ascending progressive muscle weakness, autonomic dysfunction and areflexia It causes significant morbidity requiring long hospital stay and significant period of rehabilitation Approximately 10-15% require long term residual disability assistance

11. Epidemiology Worldwide incidence is 1.1 – 1.8 cases per 100,000/year M> F Bimodal age incidence: peak in young adults and elderly > 50 yrs : incidence rises to 3.3 cases per 100,000/year Strong association(70%) with precedent respiratory and GI origin infection There is weak association between GBS and vaccination

12. Clinical features Symptoms : Weakness and sensory disturbances in LL- pain, numbness, parasthesia Progressive ascending motor weakness Respiratory mucles weakness and respiratory failure Facial nerve paralysis Bulbar weakness(involvement of CIX-CXII)- difficulty in swallowing,chewing , slurring of speech, chokking on liquids Opthalmoplegia

13. Clinical features Signs: Flaccid areflexic paralysis Muscle wasting Autonomic dysfunction Arrhythmias Swing in BP Urinary retention Paralytic ileus Hyperhidrosis

14. GBS subtypes 1) Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) 2) Acute motor axonal neuropathy(AMAN) 3) Acute motor and sensory axonal neuropathy(AMSAN) 4) Miller Fisher syndrome(MFS) 5) chronic inflammatory demyelinating polyradiculopathy (CIDP)

15. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) Most common form – 85 – 90% Symmetrical ascending motor weakness with hypo- or areflexia Underlying pathological process is inflammation and destruction of the myelin sheaths surrounding peripheral nerve axons by activated macrophages Slowing and blockage of conduction Muscle weakness Severe cases develop secondary axonal damage

16. Acute motor axonal neuropathy(AMAN) More common in Japan and China Young people Summer months Association with Campylobacter jejuni C/F similar to AIDP but tendon reflexes may be preserved Electrophysiological test distinguish from other variants – selective motor nerve and axonal involvement Binding of antibodies to ganglioside antigen on the axon Macrophages invasion, inflammation and axonal damage

17. Acute motor and sensory axonal neuropathy(AMSAN) Both motor and sensory fibers involved Severe Associated with prolonged or even partial recovery C/F are similar to AMAN but also involves sensory symptoms Pathology – antibody mediated axonal damage

18. Miller Fisher syndrome Presents with ataxia, areflexia and ophthalmoplegia 25% may develop limb weakness Electrophysiological studies – sensory conduction failure Antiganglioside antibodies to GQ1b are found in 90% of pt and are associated with opthalmoplegia Pathological – demyelination of nerve roots

19. chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) Chronic form of GBS C/F similar to AIDP Slow and progressive Or relapsing

20. Investigation Serum biochemistry : Raised ALT, GGT – 35% Raised creatinine In SIADH – deranged urea and electrolyte Inflammatory markers ESR – mostly raised CRP – sometimes raised

21. Investigation Antiganglioside and antibodies Anti-GM1 – positive in 25% ( worse outcome) Ant-GD1a – associated with AMAN subtype Ant-GQ1b – Miller-Fisher syndrome Infection screen: Campylobacter jejuni , cytomegalovirus, Epstein-Barr virus, herpes simplex virus, Mycoplasma pneumoniae, HIV

22. Investigation Radiological CT – to exclude other causes of symptoms and evidence of raised ICP MRI – may show selective anterior spinal nerve root enhancement with gadolinium Excludes cervical nerve impingement Lumbar puncture Protein – raised(may be normal in first 2 weeks) Cell count and glucose - normal

23. Investigations Nerve conduction studies Findings depend on subtype of GBS Majority show demyelinating pattern Some show axonal loss with little or no demyelination Respiratory function tests Reduced vital capacity, max inspiratory and expiratory pressure ABG – progressive respiratory failure

24. Differential diagnosis Neurological Myasthenia gravis Eaton-Lambert( myasthenic ) syndrome Multiple sclerosis Transverse myelitis Metabolic Hypokalemic periodic paralysis Hypermagnesaemia Hypophosphatasemia Acute intermittent porphyria Infective Post diphtheria neuropathy Polio botulism Tick paralysis Drugs/toxins Heavy metal poisoning Biological toxins(snake and scorpion) Drugs( stavudine , NFT, aminoglycosides) Other Acute polymyositis Critical illness myopathy

25. Diagnostic criteria for GBS, from stoelting Features required for diagnosis Progressive b/l weakness in arms and legs Areflexia Features strogy supporting the diagnosis Progression of symptoms over 2-4 wks Symmetry of symptoms Mild sensory symptoms and signs Cranial nerve involvement( esp b/l facial nerve weakness) Decrease nerve conduction velocity ANS dysfunction No fever at onset Elevated conc of protien in CSF with cell counts < 10/mm3 Spontaneous recovery starting 2-4 wks after progression halts Features making diagnosis likely Definite sensory level Marked persistent bowel and bladder dysfunction > 50 white cells/mm3 in CSF

26. Management Multi-disciplinary inputs Therapies Supportive Immunomodulatory

27. Supportive care Airway and respiratory Around 30% require ventilatory support Deterioration of respiratory function may be rapid Vital capacity provides information of about respiratory sufficiency Max inspiratory and expiratory pressure provide information about power of respective group of respiratory muscles Both test can be difficult to interpret in pts with bulbar weakness(difficulty in forming a seal around the mouth)

28. Airway and respiratory ABG – evidence of respiratory failure Desaturation can be late sign Clinical indication for intubation and ventilation are: Vital capacity of <1L or <15 mL/kg Max inspiratory pressure of < 30 cmH20 Max expiratory pressure of < 40 cm H20 Bulbar involvement with inability to cough, swallow and protect the airway Evidence of respiratory failure on ABG and autonomic instability Tracheostomy should be considered if prolonged respiratory support is needed

29. Cardiovascular Autonomic dysfunction in 70% - life threatening ECG, BP and fluid balance Most common arrhythmia – sinus tachycardia Other arrhythmias – atrial and ventricular tachycardia, prolonged QT interval, AV block, asystole BP fluctuates- severe HTN and hypotension Orthostatic hypotension is common Care should be taken when treating extremes of BP with vasoactive agents as pt may be particularly sensitive to their effects(upregulation of post synapting response) Intubated patient with autonomic dysfunction may develop instability after tracheal suction

30. Gastrointestinal Good nutrition is important particularly for those with bulbar weakness, sedated and mechanically ventilated Dietician recommendation Pt with autonomic dysfunction – paralytic ileus ( prokinetic agents – metoclopramide, erythromycin)

31. Neurological Neuropathic -50% NSAIDs in combination with opioids May add adjunctive such as anticonvulsants(Gabapentin or carbemazepine ), Tricyclic antidepressant

32. Venous thromboembolism prophylaxis High risk for DVT and PE LMWH in combination with pneumatic compression device or anti-embolism stocking recommended until pt can walk

33. Psychological High incidence of depression Counselling and psychiatric help In UK – Guillain-Barre support group

34. Rehabilitation 40% pt needs Careful attention should be paid to limb positioning and posture as limb weakness can lead to nerve compression and palsies, pressure sore and contracture Extensive input from physiotherapists and occupational therapist is essential to provide tailored strengthening exercise and support Patient may also suffer from persistent fatigue- responsive to exercise programme

35. Immunomodulatory IV immunoglobulin ( IVIg )-15 mlL /kg Its effect is comparable to plasma exchange Most effective if administered within 2 weeks of the onset of symptoms Advantages over plasma exchange: Widely available Less labour intensive Less side effect Indication: muscle weakness and respiratory depression

36. IVIg contains donor IgG antibodies – reduces severity of autoimmune inflammation in GBS by blocking Fc receptors Prevents antibody mediated cell destruction Alters complement activation Contraindication to IVIg : Previous anaphylactic reaction to IVIg and IgA deficiency Side effects of IVIg : nausea, headache, dermatological disorders(erythroderma), fluid overload, deranged LFT, venous thromboembolus , ARF, anaphylaxis No evidence of repeated treatment beneficial

37. Plasma exchange Aim – to remove antibodies associated with underlying autoimmune response Accelerates recovery Improvements have been demonstrated in regaining muscle strength, ability to walk, reduce duration of ventilator More beneficial if commenced within week of the onset of symptoms But can be beneficial upto 30 days after onset of illness Indication – same as IVIg CI: coagulopathy, overwhelming sepsis, hemodynamic instability and shock Side effects: nausea, vomiting, diarrhea, fever, coagulopathy, immunosuppression, hypoglycemia

38. Role of corticosteroids No evidence that they improve recovery or affect long term prognosis

39. Prognosis Most recover fully 15% may suffer persistent disability 10% are unable to walk unaided at 1 yr Recurrence in 2-5% Mortality – 2-12% Common death – venous thromboembolism, pneumonitis, arrhythmias and complication related to dysautonomia

40. Marker of poor prognosis Age > 40 Rapid onset of symptoms Severe weakness esp if mechanical ventilation is required or marked upper limb weakness Associated with precedent diarrheal illness or campylo bacter infection Evidence of axonal damage on electrophysiological studies Lack of treatment with either plasma exchange or IVIg

41. Anesthesia consideration

42. Anesthesia consideration Aspiration risk due to to bulbar dysfunction Perioperative respiratory insufficiency due to muscle weakness (anticipate need for postoperative ventilation) Autonomic dysfunction with possible hemodynamic instability & autonomic hyperreflexia type reactions: Arrhythmias, cardiac arrest Physical stimulation can precipitate hypertension & tachycardia  Altered response to neuromuscular blocking drugs (NMBs): Succinylcholine contraindicated due to hyperkalemia risk NdMR ( nondepolarizing muscle relaxant) sensitivity ↑ risk of venous thromboembolism

43. Anesthesia consideration Goals: Minimize aspiration risk-consider prophylaxis, RSI Maximize respiratory function – avoid NMBs or reduced dose of NdMR and full reversal, secretions, pain mx) Maintain hemodynamic stability Conflicts: RSI vs avoid succinylcholine Hemodynamic stability Neurological deficits and regional techniques

44. Pregnancy consideration Uterine tone is maintained – can deliver vaginally IUGR and oligohyramniosis – LSCS Extent of disease guides for GA/RA- no prospective randomized trial Document pre-existing deficits If GA chosen: avoid sux , minimal or avoid NdMR RA – safely used even in the presence of autonomic dysfunction(Brooks et al) with SA and EA ( Alici et al)

45. Reference GUILLAIN-BARRÉ SYNDROME ANAESTHESIA TUTORIAL OF THE WEEK 238 29th August 2011 Dr Sonya Daniel ST3, Southampton General Hospital, UK Dr Richard Green( anesthesia tutorial week) https://www.anesthesiaconsiderations.com/guillain-barre-syndrome-considerations The choice of anesthesia for cesarean section in patients with Guillain Barre Syndrome – The dilemma continues Suman Arora ,NeeruSahni, Latha Y Stoelting anesthesia and co-existing disease 7 th edition p321-322

46. Thank you