Twenty-two of 29 patients with chronic sensory ataxic neuropathy showed T2-weighted magnetic resonance imaging high signal intensity in the posterior columns of the cervical spine. T2 changes reflected the degeneration of central sensory projections and localized the disease process to T-shaped dorsal root ganglion neurons. No similar abnormalities were found in sensory and sensorimotor length-dependent axonal neuropathy patients. Spinal cord magnetic resonance imaging is a useful tool to support the clinical diagnosis of primary ganglionopathy.