Galloway G, Giuliani M J, Burns D K, Lacomis D
Department of Neurology, Louisiana State University, Shreveport, USA.
Brain Pathol. 1998 Apr;8(2):247-51. doi: 10.1111/j.1750-3639.1998.tb00150.x.
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.
关于草酸尿症相关神经病变,人们知之甚少,也尚无有效的治疗方法被描述。我们报告了两名患有与草酸尿症相关的临床严重且进行性感觉运动性多发性神经病变的患者。电诊断测试和腓肠神经组织病理学显示出严重轴突丧失和脱髓鞘的证据。此外,在内神经和神经束膜血管壁、轴突圆柱体以及肌束膜血管壁内发现了双折射晶体沉积物。电子探针显微镜检查证实钙(与草酸钙一致)是晶体的主要成分。尽管此前血液透析未能改善病情,但两名患者在进行肾和肝移植后,神经病变的体征和症状均有显著改善。我们的结果证实了先前关于与草酸尿症相关的伴有草酸钙沉积的混合性轴索性和脱髓鞘性神经病变的报道。此外,我们的研究结果表明,肾和肝移植可能是治疗相关神经病变的潜在方法。