Dorsal root gangliopathia presenting with rapidly progressing sensory polyneuropathy

A 52-year-old woman developed progressive sensory polyneuropathy leading to death in 1.5 years. Electromyography and peripheral nerve biopsy had revealed severe axonal degeneration. Neuropathological examination showed involvement of all dorsal root ganglia with loss of the bipolar nerve cells, degeneration of the remaining nerve cells, Nageotte's residual nodules, and scattered lymphocytes. The posterior columns of the spinal cord and the sensory spinal roots revealed secondary loss and degeneration of the nerve fibers. The etiology is unknown but an autoimmune-mediated reaction effecting the nervous system is strongly suggested.