Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Neurology. 2011 Aug 23;77(8):784-91. doi: 10.1212/WNL.0b013e31822b00b9. Epub 2011 Aug 10.
Among the rare causes of myelopathies is primary intramedullary spinal cord lymphoma (PISCL). As PISCL is often underrecognized, delaying appropriate treatment, we sought to describe its presentation, imaging characteristics, and outcomes.
Mayo Clinic medical records, lymphoma database, and autopsies from 1996 to 2009 were searched. Inclusion criteria were clinical myelopathic presentation, intramedullary spinal cord abnormalities, and pathologically confirmed CNS lymphoma. Exclusion criteria were extramedullary lymphoma, secondary intramedullary lymphoma, or other myelopathic etiology. Clinical features, diagnostic methods, neuroimaging, treatment, and outcomes were assessed.
The 14 patients' median age at presentation was 62.5 years (range 41-82 years) and 10 were men (71%). Two had lymphoma risk factors (HIV infection 1; chronic immunosuppression postorgan transplant 1). Most had initial presumptive diagnoses of CNS demyelinating disease and definitive diagnosis of lymphoma was delayed a median of 8 months (range 1-22 months). CNS lymphoma was pathologically confirmed by biopsy (brain 6; spinal cord 4), CSF cytology (3), and autopsy (1). Most patients had multifocal, persistently enhancing lesions on spinal MRI and 8 had involvement of conus medullaris, cauda equina, or both. IV methotrexate was the initial treatment in 9 of 12 (75%) but lymphoma recurred in the majority. Half of the patients were wheelchair-dependent at 10 months and 2-year survival was 36%.
PISCL mimics other causes of myelopathy. Spinal MRI demonstrating multifocal lesions, persistent gadolinium enhancement, and conus medullaris or cauda equina involvement is characteristic. Pathologic confirmation often requires CNS biopsy. Despite chemotherapy, morbidity and mortality is high.
在罕见的脊髓病病因中，原发性脊髓内淋巴瘤（PISCL）较为少见。由于 PISCL 常被误诊，导致治疗延误，我们旨在描述其临床表现、影像学特征和结局。
检索了 1996 年至 2009 年梅奥诊所的病历、淋巴瘤数据库和尸检记录。纳入标准为有临床脊髓病表现、脊髓内异常和经病理证实的中枢神经系统淋巴瘤。排除标准为髓外淋巴瘤、继发性脊髓内淋巴瘤或其他脊髓病病因。评估了临床特征、诊断方法、神经影像学、治疗和结局。
14 名患者的中位发病年龄为 62.5 岁（范围为 41-82 岁），其中 10 名男性（71%）。2 人有淋巴瘤危险因素（HIV 感染 1 例；器官移植后慢性免疫抑制 1 例）。大多数患者最初被诊断为中枢神经系统脱髓鞘疾病，明确诊断为淋巴瘤的时间平均延迟 8 个月（范围为 1-22 个月）。通过活检（脑 6 例；脊髓 4 例）、CSF 细胞学（3 例）和尸检（1 例）证实了中枢神经系统淋巴瘤。大多数患者的脊髓 MRI 显示多灶性、持续增强病变，8 例患者的圆锥和马尾受累或同时受累。12 例中的 9 例（75%）初始治疗采用 IV 甲氨蝶呤，但大多数患者的淋巴瘤复发。半数患者在 10 个月时需要坐轮椅，2 年生存率为 36%。
PISCL 类似于其他脊髓病的病因。脊髓 MRI 显示多灶性病变、持续钆增强、圆锥和马尾受累具有特征性。病理证实通常需要中枢神经系统活检。尽管采用化疗，发病率和死亡率仍较高。
