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A previously reported patient presenting sensory‐dominant neuropathy with antiganglioside antibodies, bound preferentially to polysi‐ alogangliosides including GD1b, was autopsied. While axonal degeneration was predominant in the sural nerve, many demyelinated fibers were present in the spinal roots. Dorsal roots had undergone significant damage. These pathological findings were well correlated with the electrophysiological results showing decreased F‐wave conduction velocities and conduction blocks in motor nerves and decreased or absent sensory action potentials in sensory nerves, with distribution of GD1b in nerve tissues such as dorsal root ganglia and paranodal myelin in the ventral and dorsal roots. © 1999 John Wiley & Sons, Inc. Muscle Nerve 22: 1426–1431, 1999