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CME

Guillain-Barre Syndrome

General Information

Description

  • Guillain-Barre syndrome is a rare autoimmune acute polyradiculoneuropathy, usually following an infection or other immune-stimulating event and presenting with bilateral weakness in the distal lower limbs, that progresses over days-to-weeks to potentially life-threatening severity requiring mechanical ventilation before plateauing for up to several months and then improving1,2

Also called

  • GBS
  • Landry-Guillain-Barre syndrome
  • Guillain-Barre-Strohl syndrome
  • idiopathic polyneuritis
  • acute inflammatory polyneuropathy
  • acute autoimmune neuropathy
  • acute inflammatory demyelinating polyneuropathy (AIDP) (most common type of GBS)

Definitions

  • acute phase of Guillain-Barre syndrome (GBS) - phase during which symptoms get progressively worse, usually taking 2-4 weeks, before improving or plateauing,2
  • nadir - period of greatest disability or muscle weakness following acute phase of GBS; may last for weeks-to-months2
  • mild GBS generally refers to being able to walk without assistance, but no standard definition (J Neurol Neurosurg Psychiatry 2017 Apr;88(4):346)

Types

  • acute inflammatory demyelinating polyneuropathy (AIDP, most common type of Guillain-Barre syndrome)1,2
  • acute motor axonal neuropathy (AMAN)1,2
    • presents with weakness without sensory symptoms or signs
    • more common in children during summer
    • more common in northern China
  • acute motor-sensory axonal neuropathy (AMSAN)1,2
    • has both weakness and sensory involvement clinically and on normal sensory studies
    • is not more common in persons of particular age groups or in particular locations
    • more protracted than AMAN and may have slower and less improvement
  • Miller-Fisher syndrome / Fisher syndrome1,2
    • is often classified as a type of acute or subacute demyelinating polyradiculoneuropathy
    • usually characterized by normal muscle strength and triad of
      • ophthalmoparesis or ophthalmoplegia
      • areflexia
      • ataxia
    • some patients also have cranial nerve or lower brainstem involvement, such as facial and pharyngeal weakness (may be referred to as Miller-Fisher-Guillain-Barre overlap syndrome)
    • some patients may present with isolated ocular nerve palsy
    • associated with anti-GQ1b antibodies in serum
    • most patients improve within 1-2 months and completely recover within 6 months without GBS-specific treatment
  • Miller-Fisher syndrome shares some clinical features with Bickerstaff brainstem encephalitis (BBE), but there is some controversy over precise diagnostic criteria and whether or not these are separate syndromes
  • paraplegic / paraparetic Guillain-Barre syndrome1,2
    • paresis restricted to the legs
    • most patients also have some upper limb sensory signs, areflexia, or electrophysiological signs
  • pharyngeal-cervical-brachial / pharyngeal-brachial Guillain-Barre syndrome1,2
  • other rare variants include
  • Study Summary
    distribution of types of Guillain-Barre syndrome varies based on region
    Details
    studySummary
    • based on population-based prospective cohort studyCohort Study
    • 925 adults aged 6 months to 88 years (mean age 51 years, 60% men) with Guillain-Barre syndrome (GBS) were followed for 1 year (from the International GBS Outcome Study [IGOS])
      • 715 patients were from Europe or the Americas (Argentina, Belgium, Canada, Denmark, France, Germany, Greece, Italy, Spain, Netherlands, United Kingdom, and United States of America)
      • 69 patients were from Asia (Japan, Malaysia, and Taiwan)
      • 125 patients were from Bangladesh
      • 15 patients were from Africa or Australia
      • remaining patients had missing data
    • comparing GBS type in patients from Europe/Americas vs. Asia (without Bangladesh) vs. Bangladesh (pairwise statistical comparisons not reported)
      • sensorimotor in 69% vs. 43% vs. 29% (p < 0.001)
      • pure motor in 14% vs. 24% vs. 69% (p < 0.001)
      • Miller-Fisher syndrome in 11% vs. 22% vs. 1% (p < 0.001)
      • pharyngo-cervical-brachial, pure sensory, ataxic, or other type in 6% vs. 11% vs. 1% (p = 0.018)
    • Brain : a journal of neurology20181001Brain1411028662866Reference - Brain 2018 Oct 1;141(10):2866PDF

References

General references used

  1. Donofrio PD. Guillain-Barré Syndrome. Continuum (Minneap Minn). 2017 Oct;23(5, Peripheral Nerve and Motor Neuron Disorders):1295-1309
  2. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016 Aug 13;388(10045):717-27
  3. Hughes RA, Wijdicks EF, Barohn R, et al. Practice parameter: immunotherapy for Guillain-Barre syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003 Sep 23;61(6):736-40full-text, reaffirmed 2016 Apr

Recommendation grading systems used

  • American Academy of Neurology (AAN) 2003 grading system for recommendations
    • levels of recommendation
      • Level A - established as effective, ineffective, or harmful or useful/predictive or not useful/predictive
      • Level B - probably effective, ineffective, or harmful or useful/predictive or not useful/predictive
      • Level C - possibly effective, ineffective, or harmful or useful/predictive or not useful/predictive
      • Level U
        • data conflicting or inadequate
        • treatment, test, or predictor unproven
    • classification of studies
      • Class I - high-quality controlled randomized trials
      • Class II - prospective matched group cohort trials or randomized trials with restrictions including
        • lacking adequate randomization concealment
        • lacking blinding
        • high dropout rate
        • bias
      • Class III - other studies including natural history studies
      • Class IV - uncontrolled studies, case series, or expert opinion
    • Reference - Practice parameter: immunotherapy for Guillain-Barre syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology (14504313Neurology 2003 Sep 23;61(6):736), reaffirmed 2016 Apr, commentary can be found in 15136711Neurology 2004 May 11;62(9):1653, mnh15122839tmdc15122839tACP J Club 2004 May-Jun;140(3):76
  • American Academy of Neurology (AAN) 2011 grading system for recommendations
    • levels of evidence
      • Level A
        • established as effective, ineffective, or harmful or established as useful/predictive or not useful/predictive for given condition in specified population (requires at least 2 consistent Class I studies)
        • in exceptional cases, 1 convincing Class I study may suffice for an "A" recommendation if
          • all quality criteria are met
          • magnitude of effect is large (relative rate improved outcome > 5 and lower limit of confidence interval is > 2)
      • Level B - probably effective, ineffective, or harmful or probably useful/predictive or not useful/predictive for given condition in specified population (requires at least 1 Class I study or at least 2 consistent Class II studies)
      • Level C - possibly effective, ineffective, or harmful or possibly useful/predictive or not useful/predictive for given condition in specified population (requires at least 1 Class II study or at least 2 consistent Class III studies)
      • Level U - data inadequate or conflicting; given current knowledge, treatment (test, predictor) is unproven (studies not meeting criteria for Class I – Class III)
    • classifications of studies
      • Class I study
        • randomized, controlled clinical trial with masked or objective outcome assessment in a representative population
        • relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences
        • all quality criteria are met
          • a) concealed allocation
          • b) primary outcome(s) clearly defined
          • c) exclusion/inclusion criteria clearly defined
          • d) adequate accounting for dropouts (with at least 80% of enrolled subjects completing the study) and crossovers with numbers sufficiently low to have minimal potential for bias
          • e) for noninferiority or equivalence trial claiming to prove efficacy for 1 or both drugs, also requires
            • authors explicitly state clinically meaningful difference to be excluded by defining threshold for equivalence or noninferiority
            • standard treatment used in study is substantially similar to that used in previous studies establishing efficacy of standard treatment (that is, for a drug, mode of administration, dose, and dosage adjustments are similar to those previously shown to be effective)
            • inclusion and exclusion criteria for patient selection and the outcomes of patients on the standard treatment are comparable to those of previous studies establishing efficacy of standard treatment
            • interpretation of results of study is based upon per protocol analysis that takes into account dropouts or crossovers
      • Class II study
        • either of
          • randomized controlled clinical trial of intervention of interest in representative population with masked or objective outcome assessment that lacks 1 criteria (a-e) above
          • prospective matched cohort study with masked or objective outcome assessment in representative population that meets b-e above
        • relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences
      • Class III study - all other controlled trials (including well-defined natural history controls or patients serving as own controls) in a representative population, where outcome is independently assessed, or independently derived by objective outcome measurement that is that is unlikely to be affected by an observer’s (patient, treating physician, investigator) expectation or bias (such as blood tests, administrative outcome data)
      • Class IV study - studies not meeting Class I, II, or III criteria including consensus or expert opinion
    • References
  • American Society for Apheresis (ASFA) recommendation grading system
    • categories of indications for therapeutic apheresis
      • Category I - disorders for which apheresis is accepted as first-line therapy, either as primary stand-alone treatment or in conjunction with other modes of treatment
      • Category II - disorders for which apheresis is accepted as second-line therapy, either as stand-alone treatment or in conjunction with other modes of treatment
      • Category III - optimum role of apheresis therapy is not established and decision-making should be individualized
      • Category IV - disorders in which published evidence demonstrates or suggests apheresis to be ineffective or harmful; Institutional Review Board (IRB) approval is desirable if apheresis treatment is undertaken in these circumstances
    • grades of recommendations
      • Grade 1A - strong recommendation, high-quality evidence
        • can apply to most patients in most circumstances without reservation
        • supported by randomized controlled trials (RCTs) without important limitations or overwhelming evidence from observational studies
      • Grade 1B - strong recommendation, moderate-quality evidence
        • can apply to most patients in most circumstances without reservation
        • supported by RCTs with important limitations (inconsistent results, methodological flaws, indirect, or imprecise) or exceptionally strong evidence from observational studies
      • Grade 1C - strong recommendation, low- or very low-quality evidence
        • recommendation may change when higher quality evidence becomes available
        • supported by observational studies or case series
      • Grade 2A - weak recommendation, high-quality evidence
        • best action may differ depending on circumstances of patients' or societal values
        • supported by RCTs without important limitations or overwhelming evidence from observational studies
      • Grade 2B - weak recommendation, moderate-quality evidence
        • best action may differ depending on circumstances of patients' or societal values
        • supported by RCTs with important limitations (inconsistent results, methodological flaws, indirect, or imprecise) or exceptionally strong evidence from observational studies
      • Grade 2C - weak recommendation, low- or very low-quality evidence
        • very weak recommendation; other alternatives may be equally reasonable
        • supported by observational studies or case series
    • Reference - ASFA guideline on use of therapeutic apheresis in clinical practice (27322218J Clin Apher 2016 Jun;31(3):149)

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National Library of Medicine, or "Vancouver style" (International Committee of Medical Journal Editors):

  • DynaMed [Internet]. Ipswich (MA): EBSCO Information Services. 1995 - . Record No. T116758, Guillain-Barre Syndrome; [updated 2018 Nov 30, cited place cited date here]. Available from https://www.dynamed.com/topics/dmp~AN~T116758. Registration and login required.

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