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孤立性动眼神经麻痹作为多发性骨髓瘤的首发表现。

Isolated third cranial nerve palsy as the first presentation of multiple myeloma.

作者信息

Vaid Tejasvini, Dhawan Rishi, Aggarwal Mukul, Tyagi Seema

机构信息

Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, India.

Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, India

出版信息

BMJ Case Rep. 2021 Mar 24;14(3):e239917. doi: 10.1136/bcr-2020-239917.

Abstract

A 50-year-old woman presented with a right-sided isolated third cranial nerve palsy. MRI brain showed a mass lesion arising from the right clivus with extension into the cavernous sinus. Blood investigations and bone marrow biopsy were suggestive of multiple myeloma with hypercalcaemia and renal dysfunction. It was unclear at first if the intracranial lesion was due to myelomatous involvement or a separate disease entirely. The patient declined consent for a biopsy and cerebrospinal fluid analysis was inconclusive. She was treated with bortezomib based chemotherapy and the palsy resolved by day 6, which helped clinch the rare diagnosis of central nervous system (CNS) involvement by multiple myeloma. Most patients with CNS myeloma have a dismal survival of under 6 months but she is on therapy for relapse 26 months after diagnosis. While placed under the umbrella of CNS myeloma, patients with osteodural myeloma have better outcomes, perhaps due to their distinct aetiopathogenesis.

摘要

一名50岁女性出现右侧孤立性动眼神经麻痹。脑部MRI显示有一肿块病变,起源于右侧斜坡并延伸至海绵窦。血液检查和骨髓活检提示为多发性骨髓瘤伴高钙血症和肾功能不全。起初尚不清楚颅内病变是由于骨髓瘤累及还是完全由另一种疾病所致。患者拒绝接受活检,脑脊液分析结果不明确。她接受了基于硼替佐米的化疗,第6天时麻痹症状消失,这有助于确诊多发性骨髓瘤累及中枢神经系统(CNS)这一罕见诊断。大多数中枢神经系统骨髓瘤患者的生存期不足6个月,但她在诊断后26个月仍在接受复发治疗。虽然归类于中枢神经系统骨髓瘤,但骨硬膜骨髓瘤患者的预后较好,这可能归因于其独特的病因发病机制。

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本文引用的文献

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Multiple myeloma with central nervous system relapse.多发性骨髓瘤伴中枢神经系统复发。
Haematologica. 2020 Jul;105(7):1780-1790. doi: 10.3324/haematol.2020.248518. Epub 2020 May 15.
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Central nervous system myelomatosis: review of the literature.中枢神经系统骨髓瘤病:文献综述
Eur J Haematol. 2008 Jan;80(1):1-9. doi: 10.1111/j.1600-0609.2007.00956.x. Epub 2007 Oct 23.

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