Abstract
We reported a 3-year-old boy of an acute axonal form of Guillain-Barre syndrome (GBS) with autoantibody against GD1b ganglioside.
A week after an episode of upper respiratory infection, he was hospitalized with a weakness in the lower extremities. Neurological examination revealed facial weakness, ristricted extra-ocular movement, hyporeflexia and tetraparesis without any sensory impairment. CSF protein was elevated with normal cell count after the first week of symptoms. Electrophysiological studies demonstrated normal motor nerve conduction velocity and low M potential amplitude. He had been on a respirator for two months. Although he received plasmapheresis in the acute phase, sixteen month after the onset he continued to have distally dominant limb weakness with wasting. Thin-layer chromatogram with immunostaining revealed that serum IgG from this patient reacted with GD1b, but did not react with GMI. Enzyme-linked immunosorbent assay showed that anti-Gun titer (IgG) decreased concurrently with the clinical improvement. There have been several reports of an acute axonal form of GBS with antibody to GM1. GD1b, as well as GMI, seems to be a target pathogenic antigen in motor axon disorders.
