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Guillain-Barre syndrome (GBS)

An acute inflammatory demyelinating polyradiculopathy described in 1916 by Guillain, Barre & Strohl. Paresthesias are the earliest symptom of Guillain Barre syndrome (GBS). Progressive symmetric ascending weakness & areflexia occurs subsequently. Dyspnea rapidly progressing to respiratory failure is s concern. [11] Since the near elimination of poliomyelitis, GBS is the most common cause of acute generalized paralysis in developed countries. Etiology: 1) cause unknown: ref 2 classifies GBS as immune-mediated 2) precipitating factors a) infection - antecedent respiratory or GI infection in 60% - within 4 weeks of symptom onset (2-4 weeks) [10] - viral - Cytomegalovirus - Epstein-Barr virus - Herpes virus: simplex & zoster - influenza A - measles - mumps - rubella - ECHO virus - Coxsackie virus - Hepatitis B - HIV1 - Zika virus - bacterial - Mycoplasma - Helicobacter pylori - Borrelia burgdorferi - Campylobacter - IgM against ganglioside GM-1 - 20% of patients with GBS - associated with axonal form & poorer prognosis - most commonly identified trigger [2,9] b) surgery: spinal anesthesia (5-10% of GBS) c) vaccines - rabies vaccine - 1976 swine influenza vaccine - oral polio vaccine 3) associated disorders a) lymphoma: Hodgkin's & non-Hodgkin's b) pregnancy & postpartum period c) systemic lupus erythematosus Epidemiology: 1) incidence: 1.7/100,000/year 2) more common in women than men 3) most common ages 50-80 Pathology: - acute autoimmune inflammatory demyelinating polyradiculoneuropathy [2] Clinical manifestations: 1) distal paresthesias (earliest manifestation) - progressive dyspnea, distal paresthesias, diminished reflexes 2) mean time to clinical function nadir (maximal symptoms) is 12 days, 98% within 4 weeks [10] - plateau phase of persistent, unchanging symptoms follows the nadir - recovery usually begins 2-4 weeks after the nadir - mean time to clinical recovery is 200 days [10] 3) ascending symmetric weakness a) upper & lower extremities b) cranial nerve involvement (45-75%) - cranial nerves 1,2 & 8 spared - facial paralysis common on initial presentation (may mimic Bell palsy) - described as difficulty holding air in cheeks [11] - diplopia, ophthalmoplegia, pupillary disturbances - dysarthria, dysphagia c) muscles of respiration - respiratory failure 30% d) rapidly progressive ascending flaccid muscle weakness [2] e) may present as acute, generalized paralysis [2] 4) pain - low back pain due to inflammatory demyelination of spinal nerve roots is common [2] - most severe in the shoulder girdle, back, buttocks, & thighs - may occur with even the slightest movements - pain is often described as aching or throbbing in nature [10] - dysesthesias in 50% with indefinate persistence in 5-10% - frequently are described as burning, tingling, or shocklike sensations - more prevalent in the lower extremities than in the upper extremities 5) hyporeflexia or areflexia 6) autonomic dysfunction 65% a) cardiac arrhythmias - tachycardia b) blood pressure fluctuation c) diaphoresis d) pupillary dysfunction e) GI dysfunction f) urinary retention g) flushing h) abnormal hemodynamic responses to pharmaceutical agents 7) minimal sensory involvement 8) antecedent infection, surgery, vaccine, or lymphoproliferative disorder - within 4 weeks of symptom onset (2-4 weeks) [10] 9) variant presentation accounts for 15% of GBS a) Fisher's syndrome (Miller-Fisher variant) b) pure motor weakness; no sensory disturbance c) isolated weakness of arm & oropharynx d) isolated weakness of leg e) facial weakness (symmetric), distal paresthesias f) ataxia & sensory loss g) autonomic neuropathy, may include sensory features h) axonal: near complete paralysis with excitable motor neurons Laboratory: - cerebrospinal fluid (CSF) analysis* a) protein > 55 mg/dL (high protein) b) if protein > 250 mg/dL, suspect spinal cord compression c) Miller Fisher syndrome ha lower CSF protein [12] d) WBC <10/mm3 (normal cell count) e) normal opening pressure f) normal in 50% in first week - neurofilament light chain in serum may be a prognostic biomarker in GBS [8] * pulmonary function testing takes priority if dyspnea on initial presentation Special laboratory: - pulmonary function testing if dyspnea or dyspnea on exertion* - vital capacity, maximal inspiratory pressure (flow-volume loop) - elctromyography & nerve conduction studies a) slowed nerve conduction velocity consistent with peripheral nerve demyelination is the most sensitive & specific test for GBS b) conduction block * catastrophic respiratory failure can occur quickly Differential diagnosis: 1) poliomyelitis a) purely motor b) asymmetric c) areflexia 2) acute myelopathy a) sensorimotor paralysis below affected spinal cord level b) sphincter paralysis 3) 'locked-in' syndrome 4) myasthenia gravis a) no sensory symptoms b) deep tendon reflexes (DTR) preserved 5) botulism a) bradycardia b) loss of pupillary reflexes c) descending flaccid paralysis 6) tick paralysis a) no sensory loss b) normal CSF protein 7) transverse myelitis Complications: - catastrophic respiratory failure can occur quickly Management: 1) plasmapheresis: 200 mL/kg of plasma removed in 4-6 sessions - reduces need for mechanical ventilation [2] 2) intravenous immune globulin (IVIG) 400 mg/kg/day [6] - ~20% or patients die or have residual disability despite immunotherapy [7] - sequential plasmapheresis, then IVIG not better than either alone [2] 3) glucocorticoids contraindicated (not beneficial, may slow recovery) 4) respiratory care a) ability to protect airway - consider tracheostomy b) ventilatory muscle weakness - assess need for endotracheal intubation c) chest physiotherapy for bedridden patients 5) supportive care a) hemodynamic monitoring as indicated b) DVT prophylaxis c) pain management d) physical therapy - orthotics & physical therapy for perisitent foot drop [11] 6) discharge to rehabiliation after stabilization [2] 7) psychological support - Guillain-Barre Syndrome Foundation International 8) vaccination appears to be reasonably safe in patients with Guillain-Barre Syndrome after vaccination [3] Prognosis: 1) generally self-limited 2) mortality: 3% a) cardiac arrest b) adult respiratory distress syndrome (ARDS) c) pulmonary embolism d) infection 3) severe residual disability 10% 4) recurrence 3% 5) poor prognosis associated with: a) advanced age b) need for ventilatory support c) electromyogram findings suggesting severe disease d) antecedent diarrhea [10]

Related

chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Specific

Fisher syndrome (Miller-Fisher syndrome, anti GQ1b antibody syndrome)

General

spinal cord disease; myelopathy (disease/disorder primarily affecting spinal cord) syndrome inflammatory demyelinating polyneuropathy

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 1073-75
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 16. 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2012, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. Baxter R et al. Recurrent Guillain-Barre syndrome following vaccination. Clin Infect Dis 2012 Mar 15; 54:800 PMID: 22267712
  4. Raphael JC, Chevret S, Hughes RA, Annane D Plasma exchange for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2012 PMID: 22786475
  5. Pritchard J. Guillain-Barre syndrome. Clin Med. 2010 Aug;10(4):399-401. PMID: 20849020
  6. Hughes RA, Swan AV, Raphael JC et al Immunotherapy for Guillain-Barre syndrome: a systematic review. Brain. 2007 Sep;130(Pt 9):2245-57. Epub 2007 Mar 2. PMID: 17337484
  7. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barre syndrome. Lancet. 2016 Aug 13;388(10045):717-27. Review. PMID: 26948435
  8. George J Blood Biomarker Predicts Guillain-Barre Outcomes - Neurofilament light may help monitor disease severity, help improve prognosis. MedPage Today November 13, 2020 httpi://www.medpagetoday.com/neurology/generalneurology/89669 - Martin-Aguilar L, et al Serum neurofilament light chain predicts long-term prognosis in Guillain-Barre syndrome patients. J Neurol Neurosurg Psychiatry 2020 PMID: 33154183 https://jnnp.bmj.com/content/early/2020/11/05/jnnp-2020-323899 - Jacobs BC Neurofilament light chain as biomarker for axonal damage in Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry 2020 PMID: 33154185 https://jnnp.bmj.com/content/early/2020/11/05/jnnp-2020-324308 - Nackerdien Z Serum NfL Levels Predict Long-Term Prognosis for Guillain-Barre Syndrome. MedPage Today 2020-12-03 https://www.medpagetoday.org/neurology/generalneurology/90002
  9. Halpin AL, Gu W, Wise ME et al Post-Campylobacter Guillain Barre Syndrome in the USA: secondary analysis of surveillance data collected during the 2009-2010 novel Influenza A (H1N1) vaccination campaign. Epidemiol Infect. 2018 Oct;146(13):1740-1745. PMID: 29986777 PMCID: PMC6123263 Free PMC article
  10. Lutsep HL Fast Five Quiz: Guillain-Barre Syndrome Practice Essentials Medscape. Dec 6, 2022 https://reference.medscape.com/viewarticle/984673
  11. NEJM Knowledge+ Neurology
  12. Al-Hakem H et al. Cerebrospinal fluid findings in relation to clinical characteristics, subtype, and disease course in patients with Guillain-Barre syndrome. Neurology 2023 Apr 19; 10.1212/WNL.0000000000207282; [e-pub]. PMID: 37076309 https://n.neurology.org/content/100/23/e2386
  13. NINDS Guillain-Barre Syndrome Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Guillain-Barr%C3%A9-Syndrome-Information-Page