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| 椎动脉夹层致双侧颈髓梗死合并意义未明单克隆免疫球蛋白血症1例报告及文献分析 |
| Bilateral cervical cord infarction due to spontaneous vertebral artery dissection complicated with monoclonal gammopathy of undetermined significance: A case report and literature review |
| 投稿时间：2025-03-07 修订日期：2025-03-07 |
| DOI： |
| 中文关键词: 脊髓梗死 椎动脉夹层 MGUS 高凝状态 |
| 英文关键词: spinal cord infarction, vertebral artery dissection, MGUS, hypercoaguability |
| 基金项目:国家自然科学基金(82101541) |
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| 摘要点击次数: 42 |
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| 中文摘要: |
| 目的：自发性椎动脉夹层常合并后循环缺血或梗死，引起孤立性脊髓梗死的情况罕见。恶性疾病相关高凝状态常引起系统性静脉或动脉血栓。意义未明单克隆免疫球蛋白血症（MGUS）是多发性骨髓瘤的癌前病变，MGUS增加体内血栓风险。现汇报椎动脉夹层致双侧颈髓梗死合并MGUS 1例，旨在提升对脊髓梗死病因的认识，避免漏诊、误诊。
方法：收集2021年1月就诊于我院神经内科的1例脊髓梗死患者的临床资料，完善血液生化检查、颈髓MRI、血管成像、骨髓活检等，并结合文献进行回顾性分析。
结果：1例68岁男性，主诉”右侧肢体无力”入院。患者骑摩托车急转弯后突发颈部放射痛，随后逐渐出现右侧肢体瘫痪、左侧肢体麻木无力及尿潴留。神经系统查体符合脊髓前动脉综合征。患者入院查D二聚体异常升高，伴左下肢肌间静脉血栓、右肺下叶肺动脉栓塞，提示患者存在高凝状态。脊髓MRI平扫及扩散加权成像证实C2-C4节段不对称性梗死。脑血管造影检查提示右侧椎动脉C3椎体层面闭塞，闭塞近端呈多发狭窄；高分辨MRI发现右侧椎动脉V2-V4段存在壁内血肿，符合椎动脉夹层表现。免疫固定电泳发现κ型IgG单克隆免疫球蛋白条带，骨髓穿刺活检显示单克隆浆细胞比例增高（3.8%），符合MGUS诊断。对该患者给予利伐沙班抗凝治疗和康复治疗，血液科建议定期随访。文献检索到椎动脉夹层所致的脊髓梗死60例，对其中34例孤立性脊髓梗死患者的临床特征进行分析发现，70.6%的患者为男性，38.2%的患者存在按摩、转颈等诱因，94.1%的患者起病时有颈部疼痛及肢体乏力等症状，82.4%的患者为单侧椎动脉夹层（其中单侧脊髓梗死占46.4%，双侧脊髓梗死占42.9%），94.1%的患者接受了抗凝或双重抗血小板治疗。检索到恶性肿瘤相关的脊髓梗死61例，其中33例为血液系统恶性肿瘤，明确考虑致病机制为肿瘤相关高凝状态的脊髓梗死有4例。1例考虑病因为椎动脉夹层的颈髓梗死患者，同时存在M蛋白血症，考虑合并MGUS。
结论：当孤立性脊髓梗死作为椎动脉夹层的非典型临床表现出现时，系统性筛查导致脊髓梗死的其他少见病因是必要的，需警惕恶性增殖性疾病尤其是血液系统疾病导致的高凝状态。 |
| 英文摘要: |
| Object: Spontaneous vertebral artery dissection (VAD), which often caused posterior circulation stroke, rarely caused isolated spinal cord infarction. Malignancy-associated hypercoaguability often caused systemic venous or arterial thromboembolism. Monoclonal gammopathy of undetermined significance (MGUS), a precursor state of multiple myeloma, can be linked to clinically significant thrombotic events. Here, we reported a case of bilateral cervical cord infarction due to spontaneous vertebral artery dissection complicated with acquired hypercoagulable state secondary to MGUS, aiming to improve cognition of the etiology of spinal cord infarction and to avoid misdiagnosis.
Methods: Clinical data of the patient, who presented at department of neurology at January 2021 and was diagnosed with spinal cord infarction, were collected. Routine blood biochemical test, cervical spinal MRI, vascular imaging and bone marrow analysis were performed. Moreover, we conducted comprehensive review of the related literature.
Results: A healthy 68-year-old man presented with acute right-sided body paralysis, accompanied by weakness and numbness of left limbs and urinary retention. Neurologic examination revealed anterior spinal artery syndrome. High blood D-dimer level and comorbidity of deep venous thrombosis and pulmonary embolism indicated coexistence of hypercoagulable state in this patient. Cervical spine MRI showed bilateral asymmetrical infarction at the C2-C4 level with diffusion restriction. Digital subtraction angiography of right vertebral artery revealed multiple stenosis followed by occlusion at C3 level and intramural hematoma in fat-suppressed T1 weighted MRI demonstrated dissection of right vertebral artery. Further hematological examination demonstrated the diagnosis of MGUS after identification of a monoclonal immunoglobulin band of IgG Kappa isotype by serum immunofixation electrophoresis and increased proportion of monoclonal plasma cells revealed by bone marrow biopsy. The patient received anticoagulation with rivaroxaban and rehabilitation therapy. The hematologist advised regular follow-up vistits. Thorough review of the literature retrieved 60 cases of spinal cord infarction due to VAD. Among the 34 cases of isolated spinal cord infarction, 70.6% were male, 38.2% with experience of massage or rotation of the neck, 94.1% presenting with neck pain and/or limb weakness, 82.4% with unilateral VAD（46.4% were unilateral cervical cord infarction，42.9% were bilateral cervical cord infarction）, 94.1% accepted anticoagulation or dual anti-platelet therapy. 61 cases of tumor associated spinal cord infarction were reported, including 33 cases of hematological malignance. Among them, 4 cases were pathophysiologically attributed to malignancy associated hypercoaguability. One case of spinal cord infarction considered to be caused by VAD and complicated with MGUS was reported.
Conclusion: Comprehensive screening of other etiologies, such as hypercoaguability secondary to hematologic malignancies is needed while spinal cord infarction occurs as an unusual complication of VAD. |
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