多发性骨髓瘤相关单侧动眼神经麻痹一例报告并文献分析

Clinical features of unilateral oculomotor nerve palsy related to multiple myeloma：a case report and literature review

目的了解多发性骨髓瘤（MM）相关单侧动眼神经麻痹的临床特点。方法我院于2013年11月临床诊断MM相关单侧动眼神经麻痹女性患者1例，年龄79岁，病史3年，本次就诊为复发，症状为单侧动眼神经麻痹，骨髓穿刺检查为骨髓瘤细胞占4.5%，血λ轻链18 g/L，CT和MRI检查示蝶鞍左侧病灶，诊断为MM IgGλ型ⅢA期，MM髓内髓外复发。结合在PubMed和清华同方期刊全文数据库1990年以来报道的MM相关单侧动眼神经麻痹病例10例，进行临床资料总结分析。结果11例中，男性7例，女性4例，发病年龄40-79（60±12）岁。IgG型7例，IgA型2例，IgG及IgA双克隆型1例，IgD型1例。9例动眼神经麻痹为MM初发的首发症状，1例为MM复发首发症状，1例出现于治疗MM过程中。7例为单纯动眼神经麻痹，4例合并滑车或外展或三叉神经等其他颅神经损害。9例影像学上发现颅底部蝶窦旁占位病灶，2例表现为MM并骨髓瘤性脑膜炎。除2例骨髓瘤性脑膜炎治疗效果较差外，其余患者在化疗和/或放疗后动眼神经麻痹体征改善明显，尤其本院病例在应用MM新型化疗药物硼替佐米治疗后动眼神经麻痹体征明显改善，且起效很快。结论对不明原因单侧动眼神经麻痹，应考虑MM可能，明确诊断后尽早治疗，硼替佐米等化疗药治疗MM相关动眼神经麻痹疗效较好。

Objective To analyze the clinical features of unilateral oculomotor nerve palsy caused by multiple myeloma（MM）. Methods A 79-year-old female diagnosed MM for 3 years was admitted due to the disease relapse. The patient presented left oculomotor nerve palsy, bone marrow biopsy showed 4.5% of myeloma cells, the serum level of λ light chain was up to 18 g/L, and brain CT/MRI scan revealed left parasphenoidsinus lesion. A diagnosis of relapsing intramedullary and extramedullary MM, IgGk type, stage IlIA was made. Ten cases were retrieved from literature since 1990, the clinical features of 11 eases were analyzed as follows. Results Among 11 cases of unilateral oculomotor nerve palsy caused by MM, 7 were males and 4 females with a mean age of （60 ± 12） years （40 -79 years）. The sub-types of MM were IgG type （7 cases）, IgA type （ 2 cases ）, biclonal of IgG and IgA type （ 1 case ）, and IgD type （ 1 case ）. Oculomotor nerve palsy was presented as the initial manifestation of MM in 9 cases, as a sign of relapse of MM in 1 case, and during MM treatment in 1 case. In 7 cases, oculomotor nerve was the only cranial nerve involved, while in other 4 cases, the Ⅳ, Ⅴ, Ⅵ cranial nerves were also involved. Neuroimaging revealed parasphenoid sinus lesions in 9 cases, and myeloma meningitis in 2 cases. Most of the reviewed eases achieved significant clinical improvement after chemotherapy and/or radiotherapy, except 2 cases with myeloma meningitis. The case in our department was improved significantly and rapidly after receiving chemotherapy with bortezomib. Conclusion MM should be considered when unilateral oculomotor nerve palsy is presented with or without the history of MM, the disease is sensitive to chemotherapy in most circumstances.