Dalolio M, Lucarella F, Rampini P, Bulfamante G P, Aldea S, Graveleau P, Gaillard S, Scarone P
Neurosurgery, department of neurological sciences, Università degli Studi di Milano, Fondazione IRCSS Cà Granda - Ospedale Maggiore Policlinico, 20122 Milan, Italy.
Unit of human pathology, department of health sciences, San Paolo hospital medical school, 20100 Milan, Italy.
Neurochirurgie. 2017 Sep;63(4):314-319. doi: 10.1016/j.neuchi.2016.11.110. Epub 2017 Sep 4.
Osteoarticular manifestations of beta-2 microglobulin amyloidosis are often diagnosed in long-term dialyzed patients. However, spinal involvement is rare (10-25% of patients), and generally not associated with neurological deterioration. Compression of the spinal cord or roots is extremely rare, and probably under-recognized.
The authors describe three cases of spinal stenosis presenting with neurological signs in long-term dialyzed patients, prospectively collected over 2 years in two different institutions and treated by surgical decompression. In all three cases, the main cause of neural compression was amyloid deposition in the spine, either extradurally in the ligamentum flavum or intradurally.
All patients improved after surgery and did not present any postoperative complications. However, two out of three patients with amyloid in the cervical spine required surgical revision to obtain a satisfactory decompression of the spinal cord.
The authors discuss spinal amyloidosis which is a well-known complication of long-term dialysis. However, neurological complications such as spinal cord or radicular symptoms have been rarely reported and, when present in dialyzed patients, are symptoms that are often attributed to other causes. To our knowledge, this is the first case series that demonstrates the relationship between neurological deterioration and amyloid depositions in the spinal canal that occur in long-term dialyzed patients. The prevalence of spinal stenosis related to the presence of amyloid in this specific subgroup of patients is probably underestimated.
β2微球蛋白淀粉样变性的骨关节表现常见于长期透析患者。然而，脊柱受累情况罕见（占患者的10% - 25%），且一般与神经功能恶化无关。脊髓或神经根受压极为罕见，可能未得到充分认识。
作者描述了3例长期透析患者出现神经症状的脊柱狭窄病例，这是在两年内前瞻性收集于两个不同机构的病例，并接受了手术减压治疗。在所有3例病例中，神经受压的主要原因是脊柱中的淀粉样蛋白沉积，沉积部位要么在硬膜外的黄韧带，要么在硬膜内。
所有患者术后均有改善，且未出现任何术后并发症。然而，3例颈椎有淀粉样蛋白沉积的患者中有2例需要进行手术翻修，以实现对脊髓的满意减压。
作者讨论了脊柱淀粉样变性，这是长期透析的一种已知并发症。然而，诸如脊髓或神经根症状等神经并发症鲜有报道，且在透析患者中出现时，这些症状常被归因于其他原因。据我们所知，这是首个表明长期透析患者神经功能恶化与椎管内淀粉样蛋白沉积之间关系的病例系列。在这一特定亚组患者中，与淀粉样蛋白存在相关的脊柱狭窄患病率可能被低估了。
