acute inflammatory demyelinating polyneuropathy (AIDP) Guillain-Barre syndrome
Conditions
Interventions
None listed
Sponsors
Erasmus MC, Universitair Medisch Centrum Rotterdam
Eligibility
Age
2 Years to 99 Years
Inclusion criteria
Inclusion criteria: • Patients are diagnosed with GBS according to NINDS diagnostic criteria.(3) • Indication to start IVIg treatment: 1. Patient is unable to walk unaided for >10 meter (grade 3, 4 or 5 of the GBS disability scale) or 2. There is otherwise an indication to start IVIg treatment according to the treating neurologist • Onset of weakness due to GBS is less than 2 weeks ago. • Signed informed consent.
Exclusion criteria
Exclusion criteria: • Age less than 6 years • Patient known to have a severe allergic reaction to properly matched blood products or plasma products. • Pregnancy or breastfeeding. • Patient known to have a selective IgA deficiency. • Patient shows clear clinical evidence of a polyneuropathy caused by e.g. diabetes mellitus (except mild sensory), alcoholism, severe vitamin deficiency, porphyria. • Patient received immunosuppressive treatment (e.g. azathioprine, cyclosporine, mycofenolatemofetil, tacrolimus, sirolimus or > 20 mg prednisolon daily) during the last month. • Patient known to have a severe concurrent disease, like malignancy, severe cardiovascular disease, AIDS, severe COPD. • Inability to attend follow-up during 6 months. Relative contra-indications for second IVIg dose: • Patients known to have severe kidney dysfunction (GFR below 40 ml/min). • Pre-existing risk factors of thrombo-embolic complications or severe ischemic heart disease.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main study endpoint GBS disability score at 4 weeks after start of first IVIg dose. The full range of scores will be considered as an ordinal outcome scale. In analyzing we will use a proportional odds model. (Extent of) improvement on this ordinal scale will be compared between groups. | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary study endpoints - Percentage of patients that improve: at least 1, 2, 3 or 4 points on the GBS disability score at 4, 8, 12 and 26 weeks, at least 4, 8 or 12 points on MRC sum score (ranging from 0-60) at 4, 8, 12 and 26 weeks, at least 2, 4 or 6 points on ONLS score (ranging from 0-12) at 4, 8, 12 and 26 weeks. - Percentage of patients needing artificial ventilation. - Time (number of days) on respirator. - Time (number of days) on intensive care unit. - Percentage of patients that die because of GBS. - Time (number of days) to hospital discharge. - Percentage of patients with secondary deterioration due to treatment-related fluctuations (TRF). - Development of complications possibly related to a second IVIg course. - Serum IgG levels at 4 different time points. Other study parameters - To correct for known prognostic factors (age, preceding diarrhea, positive serology of different micro-organisms, antibodies against gangliosides) we will ask questions about antecedent events, as diarrhea and upper respiratory tract infection and perform laboratory measurements (serology against Campylobacter jejuni, cytomegalovirus (CMV), Epstein-Barr virus (EBV), Mycoplasma pneumonia and IgG, IgM and IgA antibodies against GM1, GD1a and GQ1b). | — |
Countries
Netherlands
Outcome results
None listed