Guillain Barre’ syndrome(GBS) and Anesthesia consideration
- 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 4th 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 14th 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 4th 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
- 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
- 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 7th edition p321-322
- 46. Thank you
Editor's Notes
- #28: Sabrasez breath holding test: • Ask the patient to take a full but not too deep breath & hold it as long as possible. >25 SEC.-NORMAL Cardiopulmonary Reserve (CPR) 15-25 SEC- LIMITED CPR <15 SEC- VERY POOR CPR 2) Single breath count: After deep breath, hold it and start counting till the next breath. N- 30-40 COUNT Indicates vital capacity 3) SCHNEIDER’S MATCH BLOWING TEST: MEASURES Maximum Breathing Capacity. Ask to blow a match stick from a distance of 6” (15 cms) with- Mouth wide open Chin rested/supported No purse lipping No head movement No air movement in the room Mouth and match at the same level. Can not blow out a match MBC < 60 L/min FEV1 < 1.6L Able to blow out a match MBC > 60 L/min FEV1 > 1.6L MODIFIED MATCH TEST: DISTANCE MBC 9” >150 L/MIN. 6” >60 L/MIN. 3” > 40 L/MIN. 4) COUGH TEST: DEEP BREATH F/BY COUGH ABILITY TO COUGH STRENGTH EFFECTIVENESS INADEQUATE COUGH IF: FVC<20 ML/KG FEV1 < 15 ML/KG PEFR < 200 L/MIN. VC ~ 3 TIMES TV FOR EFFECTIVE COUGH. A wet productive cough / self propagated paraoxysms of coughing – patient susceptible for pulmonary Complication.
- #29: Initial tratment is facemask then CPAP. With CPAP the vital capacity and max inspiratory and expiratory pressure is known, if inadequate intubation