Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain-Barré syndrome

Abstract

1 and 4 while the two others had demyelinative features. All four had raised cerebrospinal fluid protein concentrations upon admission and patient 4 had a 25 000 GM1 antibody titre. The patients were retreated with IVIg after 14–21 days and substantial or rapid improvement was noted within days (figure). All four patients began to walk within several weeks. Patient 1 is of special interest because he was in a critical condition for 15 days with flaccid quadriplegia, respiratory failure, and severe autonomic instability, with long asystoles necessitating a pacemaker. Electrophysiological studies at this phase showed inexcitable nerves in all his limbs. During the second course of IVIg he began to improve and to make a remarkable recovery. Unlike the patients reported by Irani et al no relapses occurred in all 12 patients after the onset of motor improvement. As the figure suggests, it appears that the second course of IVIg had a favourable impact on the clinical course of these patients. This favourable response is especially striking as two of the patients had features compatible with an adverse prognosis (severe axonal involvement in both and antecedent diarrhoea and high titres of GM1 antibodies in one). The outcome in these patients suggests that in some GBS cases the standard dose of IVIg is not sufficient and that in such cases it is justified to repeat the treatment. This is true even in cases with adverse prognostic features such as early axonal damage. Now that it has been determined that IVIg and plasma exchange are equally effective and that the combination of therapies offers no advantage, a next logical step might be a controlled study designed to determine optimum doses and regimens of current therapies in GBS.