| 舒远禄,林翔,倪睿涵,等.慢性炎性脱髓鞘性多发性神经病合并脊髓硬脊膜动静脉瘘 1例报告及文献深度解析[J].安徽医药,2025,29(11):2180-2185. |
| 慢性炎性脱髓鞘性多发性神经病合并脊髓硬脊膜动静脉瘘 1例报告及文献深度解析 |
| A case report of CIDP combined with spinal dural arteriovenous fistula and in-depth analysis of the literature |
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| DOI：10.3969/j.issn.1009-6469.2025.11.012 |
| 中文关键词: 慢性炎性脱髓鞘性多发性神经病 脊髓 动静脉瘘 脑脊髓液蛋白质类 神经节苷脂类 甲泼尼龙 矛盾性栓塞 脊髓数字减影血管造影 |
| 英文关键词: Chronic inflammatory demyelinating polyradiculoneuropathy Spinal cord Arteriovenous fistula Cerebrospinal fluid proteins Gangliosides Methylprednisolone Paradoxical embolism Spinal cord digital subtraction angiography |
| 基金项目:国家自然科学基金项目( 81960231) |
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| 中文摘要: |
| 目的探讨 1例慢性炎性脱髓鞘性多发性神经病( CIDP)合并脊髓硬脊膜动静脉瘘( SDAVF)病人中的多因素诊疗挑战，包括潜在的遗传基因突变、易栓症可能性及免疫功能紊乱。方法全面回顾 2023年 10月贵州医科大学附属医院收治的 1例 CIDP合并脊髓 SDAVF病人的病史、实验室检测、影像学检查及治疗过程，综合分析病人的临床特征和并发症。结果男性， 70岁，汉族，因“双下肢乏力 1年，加重伴四肢麻木 1个月”入院。入院后查脑脊液蛋白 569 mg/L；抗 GM3神经节苷脂抗体( anti-GM3 ganglioside antibody，GM3 Ab)免疫球蛋白( Ig)G阳性；电生理示以脱髓鞘为主的混合性周围神经病。甲泼尼龙 500 mg冲击后次日乏力加重并出现排便、排尿困难。磁共振成像( MRI)/CT血管成像( CTA)提示 T9-T12异常；数字减影血管造影( DSA)证实 T12水平椎管内后方迂曲血管影，考虑脊髓 SDAVF，因供血动脉纤细未予栓塞，病人家属拒绝外科切除。住院期间突发左上肢无力，证实左锁骨下动脉闭塞；肺动脉 CTA见双侧多发栓塞，合并卵圆孔未闭与下肢静脉血栓，考虑矛盾性栓塞，予抗凝。出院时可拄拐行走，上肢肌力 5级、下肢 4+级； 3个月随访改良 Rankin量表( mRS)评分 2分。结论对 CIDP病人若用激素治疗后症状显著加重或出现括约器功能障碍，应高度警惕合并脊髓 SDAVF，立即行脊髓 MRI/DSA以筛查。另外，在处理复杂的神经病学病例时，需要考虑到潜在的遗传因素和多系统并发症，以做出更准确的诊断和采用有效的治疗策略。 |
| 英文摘要: |
| Objective To explore the multi-factor diagnosis and treatment challenges in a patient with chronic inflammatory demye-linating polyneuropathy (CIDP) and spinal vascular malformation, including potential genetic mutations and the possibility of thrombo-philia and immune dysfunction.Methods A comprehensive review of the medical history, laboratory tests, imaging examinations, andtreatment process of a patient with CIDP and spinal SDAVF admitted to The Affiliated Hospital of Guizhou Medical University in Octo-ber 2023, and a comprehensive analysis of the patient's clinical characteristics and complications.Results A 70-year-old male patientof Han nationality was admitted to the hospital due to weakness in both lower limbs for one year, aggravated by numbness in the limbsfor one month. Upon admission, cerebrospinal fluid protein was 569 mg/L, anti-GM3 IgG was positive, and electrophysiological findingsrevealed mixed peripheral neuropathy with demyelination as the primary symptom. A 500 mg methylprednisolone bolus was adminis-tered the following day, worsening weakness and dyspnea and urination. MRI/CTA revealed abnormalities at T9-T12. DSA confirmed tortuous posterior intraspinal vessels at the T12 level, suggesting a SDAVF. Due to the thinness of the feeding artery, embolization wasnot performed, and the family declined surgical resection. During hospitalization, he developed sudden onset of left upper limb weak-ness, confirming left subclavian artery occlusion. Pulmonary artery CTA revealed bilateral multiple emboli, combined with patent fora-men ovale and lower limb venous thrombosis, suggesting paradoxical embolism, and he was treated with anticoagulation. At discharge,he was able to walk with crutches, with upper limb strength at level 5 and lower limb strength at level 4+. At three-month follow-up, his mRS score was 2. Conclusions For CIDP patients, if symptoms significantly worsen or sphincter dysfunction occurs after hormonetreatment, high suspicion should be given for the presence of a spinal dural arteriovenous fistula, and spinal MRI/DSA should be per-formed immediately for screening. This case illustrates the need to consider underlying genetic factors and multisystem complicationswhen managing complex neurological cases to achieve more accurate diagnosis and effective treatment strategies. |
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