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Guillain-Barre Syndrome

 

Principal Investigator: Dr Malcolm Daniel

Guillain-Barre Syndrome – The effects of therapy

 

Current State: Complete,  Submitted August 2004, Published September 2004.

 

Completed Topics GBS Summary Raphael,Cochrane,04 Hughes,Cochrane,03 Hughes,Cochrane,04 Knoningsveld,2004

 

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Guillain-Barre Syndrome – The effects of therapy

 

Malcolm Daniel on behalf of the SICS EBM Group

Consultant in Anaesthesia and Intensive Care, Department of Anaesthesia, Walton Building, Glasgow Royal Infirmary, 84 Castle Street, G4 0SF. Email: md235@uclcf.gla.ac.uk

Citation: EBM Reviews. Scottish Intensive Care Society EBM Group. Daniel M. Guillain-Barre Syndrome - The Effects of Therapy. 2004.

This review focuses on the effects of treatments used for Guillain-Barre syndrome. 

 

Prevalence: This is a rare condition in the general population.   The reported incidence is between 0.5 and 2 cases per 100 000 people per year.

 

Mechanism: The cause remains under investigation.   The hypothesis is that GBS is due to an autoimmune response to antigens in the peripheral nervous system.  In about 50% of patients the condition develops after a triggering factor, usually an infection, but other triggers have been noted.

 

Prognosis: About 25% of patients with Guilllain-Barre syndrome will require ventilatory support during the course of their illness.    Recovery takes several weeks to months.   Up to 10% of patients remain unable to walk unaided a year after diagnosis.   The mortality rate for Guillain-Barre syndrome (all degrees of severity) is 5 - 10%.

 

This review looks at the effects of the three principle treatments used for Guillain-Barre syndrome:

 

·        Plasma exchange

·        Intravenous immunoglobulin

·        Steroids 

Endpoints: The most commonly reported endpoint of therapeutic trials involving patients with Guillain-Barre syndrome is an improvement in the Guillain-Barre Syndrome disability index (or other similar score) by one or more points over a four week period. 

Index

Physical state

0

Healthy

1

Minor symptoms & capable of running

2

Able to Walk 10m or more without assistance but unable to run

3

Able to walk 10m across an open space with help

4

Bedridden or chairbound

5

Needs assisted ventilation for at least part of the day

6

Dead

The available trials have been reviewed as part of the work of the Cochrane Neuromuscular Disease Group. There are three systematic reviews that have been appraised, together with one randomised trial that has been published since the most recent update to the systematic reviews. These four publications have been appraised using CATmaker™ software

 Each critically appraised topic (CAT) contains a brief (1 page) summary of the article reviewed. This includes 3 useful features:

·        The level of evidence using the current Scottish Intercollegiate Guidelines Network (SIGN) method of grading healthcare recommendations. This grading reflects only the weight of the evidence, not the clinical importance of the recommendation.

·        A web link to the full-text article or abstract is provided, depending on the journal the article was published in. This allows you to check the article too!

·        The search strategy used to find the article reviewed. This will allow repetition of the same search in the future to check if new evidence has accumulated.

·        Evidence based quality filters were also used. Evidence based quality filters select out articles of a certain publication type such as randomised controlled trials or systematic reviews or cohort studies. This helps set your search towards therapeutic interventions, diagnostic procedures or prognostic factors depending on the quality filter used.

Trish Greenhalgh’s BMJ article “How to read a paper: The Medline database” gives further useful details on how do this, together with details of search strategies that can be used.

This collection does not tell you how to manage your patient. We have produced a summary the evidence together with an index of the weight of the evidence, but only you (with your personal clinical expertise), your patient (with their ideas and values), together with knowledge of the practice environment in which you work can determine the correct path.

 Main Results:


Plasma exchange

The only treatment shown to have beneficial effect compared to standard supportive therapy alone. Grade A recommendation.

 For every 5 patients given plasma exchange, compared to standard therapy alone, one more patient has an improvement in GBS disability score by one or more at 28 days (95% CI 3 to 6).  

For every 8 patients given plasma exchange, compared to standard therapy alone, one less patients requires ventilatory support (95% CI 5 to 15).

 


Intravenous immunoglobulin

The two trials of iv immunoglobulin versus supportive therapy alone do not have sufficient data to draw a firm conclusion.   Five trials comparing immunoglobulin versus plasma exchange show no difference in effect.   Intravenous immunoglobulin has similar beneficial effect. Grade A recommendation. 

All trials report increased incidence of adverse events in those receiving plasma exchange, however, the definitions used vary between trials and it is not possible to pool the results.

Intravenous immunoglobulin is easier to give than plasma exchange (this requires specialist equipment and specialist clinical teams).


Steroids

No evidence of benefit in either the systematic review or in the most recently published multicentre randomised controlled trial.     Grade A recommendation.


References (With Links to CATs): 

  1. Plasma exchange for Guillain-Barre syndrome.    Raphael, JC; Chevret, S; Hughes, RAC; Annane, D.    Cochrane Database of Systematic Reviews 2004. Date of Most Recent Update: 12-January-2004. CAT.

  2. Intravenous immunoglobulin for Guillain-Barre syndrome.  Hughes, RAC; Raphael, JC; Swan, AV; van Doorn, PA. Cochrane Database of Systematic Reviews 2004.    Date of Most Recent Update: 26-November-2003. CAT.

  3. Corticosteroids for Guillain-Barre syndrome    Hughes, RAC; van der Meche, FGA.   Cochrane Database of Systematic Reviews 2004.   Date of Most Recent Update: 17-February-2004. CAT.

  1. Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial. R van Koningsveld, P I M Schmitz, F G A van der Meché, L H Visser, J Meulstee, P A van Doorn, for the Dutch GBS study group.    Lancet 2004; 363: 192-96. CAT.

 

NHSScotland staff are able to access the Cochrane database through OVID Medline.

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 Edited in behalf of the SICS EBM Group by CC.

 ©2004 Scottish Intensive Care Society Evidence-Based Medicine Group.