University of Michigan
Department of Pediatrics
Evidence-Based Pediatrics Web Site

Question

• An 8-year old girl presents with one week of progressive ascending motor weakness, subsequently diagnosed as Guillain-Barre syndrome.  Consideration is given to therapy with plasmapheresis or intravenous immunoglobulin.

Clinical Bottom Lines

1. Intravenous immunoglobulin (0.4 g/kg daily for 5 days) is equivalent to plasma exchange (five 50 ml/kg exchanges over 8-13 days) for treatment of Guillain-Barre syndrome in adults.¹  Therapy with both plasma exchange and immunoglobulin conferred no additional benefit.
2. Treatment with intravenous immunoglobulin led to more rapid improvement (27d vs. 41d) and resulted in significantly fewer complications (7% with multiple complications vs. 22% for plasma exchange, p<0.01).²
3. Intravenous immunoglobulin therapy is less expensive³ and is easier to administer.=

Summary of Key Evidence

1. Summarize the key evidence so that others can see it for themselves.
2. immunoglobulin, or both.¹  Inclusion criteria were severe disease (aid needed for walking), age over 16 years, and onset of disease within the past 14 days.  Patients were followed up for 48 weeks.
3. The major outcome measure was improvement on a seven-point disability scale (0=healthy without signs of disease to 6=dead).  Secondary outcomes included: time to recovery of unaided walking, time to discontinuation of ventilation, and the trend in improvement of the disability over the follow-up period.
4. 121 patients received plasma exchange (PE), 130 received immunoglobulin (IVIg), and 128 received both (PE+IVIg).
5. The mean change in disability grade after 4 weeks was 0.9, for PE, 0.8 for IVIg, and 1.1 for PE+IVIg.  Median days to unaided walking was 49 for PE and 51 for IVIg.  At 48 weeks, 16.7% were not walking after treatment with PE compared to 16.5% after treatment with IVIg.  Median days to stop artificial respiration were 29 for PE and 26 for IVIg.  The number of deaths were 5 in the PE group and 6 in the IVIg group.
6. Complications occurred in eight patients treated with PE alone and six patients treated with IVIg alone.  However, 13.8% of patients treated with PE were not able to tolerate the full treatment course compared to only 2.3% of those treated with IVIg.

Additional Comments

• No randomized studies performed in children have been published.  One small (n=7) study evaluated the use of intravenous immunoglobulin in children and compared the results to an earlier study of plasma exchange (n=8) done at the same institution.⁴  Their conclusion was that intravenous immunoglobulin is the preferred therapy.
• It seems that the adult studies should be applicable to children.  However, some investigators have suggested that the course and prognosis may be more favorable for children with Guillain-Barre syndrome than for adults.³
• Intravenous immunoglobulin therapy is thought to exert a beneficial effect by the anti-idiotypic suppression of autoantibodies.¹
• Intravenous immunoglobulin therapy has side effects including anaphylactoid reaction and aseptic meningitis; it is also costly.
• The biggest barrier to further study at the present time is the limited availability of intravenous immunoglobulin.

Citation

1. Anonymous. Randomized trial of plasma exchange, intravenous immunoglobulin, and combined treatments in GuillainBarre syndrome.  Plasma Exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group.  Lancet. 349(9407):225-30, 1997.
2. van der Meche FGA, Schmitz PIM.  A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barre syndrome.  N Engl J Med.  326(17):1123-9, 1992.
3. Vajsar J, Sloane A, Wood E, Murphy EG.  Plasmapheresis vs intravenous immunoglobulin treatment in childhood Guillain-Barre syndrome.  Archives of Pediatric & Adolescent Medicine.  148(11):1210-2, 1994.
4. Abd-Allah SA, Jansen PW, Ashwal S, Perkin RM.  Intravenous immunoglobulin as therapy for pediatric Guillain-Barre syndrome.  Journal of Child Neurology.  12(6):376-80, 1997.

CAT Author: John Frohna, MD

CAT Appraisers: <Reviewers>, MD

Date appraised: July 27, 1998