Neuropathy associated with hyperoxaluria: improvement after combined renal and liver transplantations.

摘要

Little is known about oxaluria-associated neuropathy, and no effective treatments have been described. We report two patients with clinically severe and progressive sensorimotor polyneuropathy associated with oxaluria. Electrodiagnostic testing and sural nerve histopathology revealed evidence of severe axon loss and demyelination. In addition, birefringent crystalline deposits were identified within endoneurial and perineurial blood vessel walls, axon cylinders, and perimysial blood vessel walls. Electron probe microscopy confirmed that calcium (consistent with calcium oxalate) was a major constituent of the crystals. Both patients had substantial improvement in neuropathic signs and symptoms after kidney and liver transplantations despite no prior improvement with hemodialysis. Our results confirm previous reports of a mixed axonal and demyelinating neuropathy with calcium oxalate deposition in association with oxaluria. In addition, our findings suggest that renal and liver transplantation may be potential treatments for the associated neuropathy.