INTRAVENOUS IMMUNOGLOBULIN TREATMENT IN LOWER MOTOR-NEURON DISEASE-ASSOCIATED WITH HIGHLY RAISED ANTI-GM1 ANTIBODIES

The effect of intravenous immunoglobulin (IVIg) treatment was studied in five patients with lower motor neuron disease associated with highl y raised anti-GM1 antibodies but without evidence of conduction block on neurophysiological examination. The patients received IVIg treatmen t (0.4 g/kg for five consecutive days) in an open study. Only one pati ent responded to IVIg treatment, which was confirmed in a double blind , placebo controlled study (two placebo treatments and two IVIg treatm ents in a randomised order). However, after six months of maintenance IVIg treatment (0.4 g/kg weekly) muscle weakness gradually deteriorate d below pretreatment levels despite continued treatment. It is conclud ed that the presence of raised anti-GM1 antibodies does not identify a subgroup of patients with lower motor neuron disease who respond to I VIg treatment and although some patients with lower motor neuron disea se may initially respond, IVIg treatment does not seem to be sufficien t as long term treatment.