In studies of Guillain-Barré syndrome, functional deficit is usually assessed according to a functional scale consisting of several categories. The level of interobserver agreement in this scoring method is not known; furthermore, this method seems to be insensitive when applied to bedridden and artificially ventilated patients. We have developed an additional score (MRC-sumscore), reflecting muscle strength in general. Both scoring methods, tested in Guillain-Barré patients, have an almost perfect interobserver agreement. For the functional score kappa = 0.85, and for the MRC-sumscore r2 = 0.96. The MRC-sumscore is easily assessed and more sensitive than the functional score when patients are bedridden or artificially ventilated.

No abstract

Since the introduction of plasma exchange as a treatment for GuillainBarre syndrome (GBS) patients, treatment related fluctuations have been found to occur in about 10% of the patients. These fluctuations are considered additional evidence of the beneficial effect of plasma exchange. In this report the occurrence of such treatment related fluctuations is described in the 147 patients who took part in the Dutch Guillain-Barre trial comparing high dose intravenous immunoglobulin with plasma exchange. Six of 72 patients in the plasma exchange group and eight of 74 in the immunoglobulin group showed such fluctuation. These results support the biological effect of immunoglobulin. More general use of immunoglobulin should await the full analysis of the Dutch GBS trial which is in progress.

Intravenous gammaglobulin (IVGG) can improve the clinical course of several immune-mediated diseases. We report the first results of such treatment in eight patients with severe Guillain-Barré syndrome (GBS). We observed that IVGG was beneficial in at least some of the patients with severe GBS. These results should be confirmed in a randomized trial.

One course of intravenous immunoglobulins (IVIg) of 2 g/kg is standard treatment in Guillain-Barré syndrome (GBS) patients unable to walk independently. Despite treatment some patients recover poorly, in part related to rapid consumption of IVIg, indicating that they may benefit from a second course of IVIg. The aim of the study is to determine whether a second course of IVIg, administered 1 week after start of the first course in patients with GBS and predicted poor outcome improves functional outcome on the GBS disability scale after 4 weeks. Secondary outcome measures include adverse events (AEs), Medical Research Council sumscore and GBS disability score after 8, 12, and 26 weeks, length of hospital and ICU admission, mortality, and changes in serum IgG levels. GBS patients of 12 years and older with a poor prognosis, based on the modified Erasmus GBS outcome score (mEGOS) at 1 week after start of the first IVIg course are eligible for randomization in this double-blind, placebo-controlled (IVIg or albumin) clinical trial. This study will determine if a second course of IVIg administered in the acute phase of the disease is safe, feasible, and effective in patients with GBS and a poor prognosis. This Dutch trial is registered prospectively as NTR 2224 in the Netherlands National Trial Register (NTR) which is the Primary Registry in the WHO Registry Network for the Netherlands.