Ataxic form of Guillain-Barre syndrome associated with anti-GD1b IgG antibody

Abstract

Richter1 proposed an ataxic variant of Guillain-Barré syndrome, in which patients have severe ataxia of the cerebellar type at the onset of Guillain-Barré syndrome but no ophthalmoplegia or severe loss of proprioceptive sense. Patients with ataxic Guillain-Barré syndrome have distal paraesthesias, areflexia, and raised CSF protein concentrations. Kusunoki et al 2 reported that of 149 patients who had anti-GQ1b IgG antibodies without profound weakness, five had acute self limited ataxia without ophthalmoplegia. The nosology of these patients, however, was not discussed. Of our 340 consecutive patients who had anti-GQ1b IgG, six had no external ophthalmoplegia and one had minimal external ophthalmoplegia. The clinical features of these seven anti-GQ1b-positive patients were consistent with an “ataxic form of Guillain-Barré syndrome” (Yuki et al , unpublished observations). Tentative diagnoses made by the primary physicians were Guillain-Barré syndrome (n=3), atypical Miller Fisher syndrome (n=3), and acute cerebellar ataxia (n=1). Araki et al ,3 however, reported on a patient with Guillain-Barré syndrome who had prominent cerebellar signs. That patient …