We studied the frequency, fine specificity and clinical correlate of anti-GQ1b IgG and IgM antibodies in 216 patients with neuropathy including three with Miller Fisher syndrome (MFS), 73 with Guillain-Barré syndrome (GBS), 99 with neuropathy associated with IgM monoclonal gammopathy (PN+IgM) and 41 with other neuropathies, and compared the data with 92 disease or normal controls. We found high (>1/100) anti-GQ1b IgG titers in all three MFS patients and in two GBS patients (2.7%) with ophthalmoplegia and ataxia, while high anti-GQ1b IgM were only found in two patients with a chronic demyelinating sensorimotor neuropathy associated with IgMkappa monoclonal gammopathy (2%). By overlay HPTLC, IgG antibodies in MFS and GBS either selectively reacted with GQ1b or also bound to GD3, and less intensely to GD1b, while IgM antibodies from both patients with PN+IgM also strongly reacted with GD1b and, in one, with GD3 and GT1b. The constant association of anti-GQ1b antibodies with dysimmune neuropathies and the correlation between their isotype, fine specificity and clinical presentation, support a possible pathogenetic link between these antibodies and the neuropathy.