Mizuno J, Nakagawa H, Tsuji Y, Yamada T
Department of Neurological Surgery, Aichi Medical University, Aichigun, Aichi, Japan.
Surg Neurol. 2001 Jun;55(6):378-82. doi: 10.1016/s0090-3019(01)00465-7.
Primary solitary amyloidoma of the spine is a rare disease characterized by localized deposition of amyloid. To the best of our knowledge, there have been only 14 cases previously reported in the literature. Patients with focal spinal amyloidoma usually have relatively long symptomatic periods preoperatively, ranging from 3 weeks to 6 years (mean: 12 months). Only two reported patients had acute paraplegia. We add a third case of a thoracic spine amyloidoma presenting with acute paraplegia.
A 65-year-old man presented with a three-day history of progressive paraplegia and urinary retention. He was found to have severe cord compression at T2 on magnetic resonance imaging. He underwent emergent decompressive laminectomy with instrumentation for spinal stabilization. Histopathology revealed abundant amyloid deposits. A systemic work-up was negative for amyloidosis. The patient showed marked neurological improvement with residual mild spastic gait after 1 year.
Primary spinal amyloidoma with acute paraplegia is rare. One-stage surgery combining prompt decompression and stabilization of the spinal column is mandatory in cases of spinal amyloidoma with acute myelopathy, because primary solitary amyloidoma carries a good prognosis.
脊柱原发性孤立性淀粉样瘤是一种罕见疾病，其特征为淀粉样物质的局限性沉积。据我们所知，此前文献中仅报道过14例。局灶性脊柱淀粉样瘤患者术前通常有相对较长的症状期，从3周至6年不等（平均：12个月）。仅有2例报道患者出现急性截瘫。我们补充了第3例表现为急性截瘫的胸椎淀粉样瘤病例。
一名65岁男性，有3天进行性截瘫和尿潴留病史。磁共振成像显示其T2水平存在严重脊髓受压。他接受了急诊减压性椎板切除术并植入器械以稳定脊柱。组织病理学检查显示有大量淀粉样沉积物。全身检查未发现淀粉样变性。1年后，患者神经功能明显改善，遗留轻度痉挛性步态。
原发性脊柱淀粉样瘤伴急性截瘫较为罕见。对于伴有急性脊髓病的脊柱淀粉样瘤病例，一期手术联合迅速减压和脊柱稳定术是必要的，因为原发性孤立性淀粉样瘤预后良好。
