Sensory neuropathy associated with primary biliary cirrhosis. Histologic and morphometric studies

Sensory symptoms can develop in the course of primary biliary cirrhosis. They have been attributed to xanthomatous infiltrates of the nerves. We report a case of a young woman suffering from pure sensory neuropathy starting at a time when evidence of liver disease was minimal. Histologic and morphometric studies of sural nerve biopsy specimens obtained from calf and ankle showed a dying-back type of axonal degeneration predominantly affecting large myelinated fibers. No lipid-containing cells were seen, which raises the possibility that other mechanisms related to primary biliary cirrhosis are involved in the pathogenesis of the neuropathy.