视神经脊髓炎谱系障碍作为一种副肿瘤综合征：一种罕见且具有挑战性的诊断。
Neuromyelitis optica spectrum disorder as a paraneoplastic syndrome: a rare and challenging diagnosis.
作者信息
Gibril Mukhtar, Walters Richard
机构信息
General Medicine, Morriston Hospital, Swansea, UK
Neurology Department, Morriston Hospital, Swansea, UK.
出版信息
BMJ Case Rep. 2021 May 4;14(5):e239389. doi: 10.1136/bcr-2020-239389.
Abstract
Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system, often associated with aquaporin-4 antibodies (AQP4-Ab). NMOSD may present as a subacute myelopathy, progressing over days with MRI revealing a contiguous inflammatory lesion of the spinal cord, ≥3 vertebral segments, a longitudinally extensive transverse myelitis. We describe an unusual paraneoplastic form of AQP4-Ab NMOSD that developed in a patient with an advanced diffuse large B-cell lymphoma. The patient had an unusual hyperacute onset, reaching a clinical nadir within hours.
摘要
视神经脊髓炎谱系障碍（NMOSD）是一种罕见的中枢神经系统抗体介导疾病，常与水通道蛋白4抗体（AQP4-Ab）相关。NMOSD可能表现为亚急性脊髓病，在数天内进展，MRI显示脊髓连续的炎性病变，累及≥3个椎体节段，即纵向广泛横贯性脊髓炎。我们描述了1例发生于晚期弥漫性大B细胞淋巴瘤患者的不寻常的副肿瘤性AQP4-Ab NMOSD形式。该患者起病异常急骤，在数小时内病情就发展至最低点。
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