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Bing-Neel 综合征的眼眶累及。

Orbital involvement in Bing-Neel syndrome.

机构信息

Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.

出版信息

J Neuroophthalmol. 2010 Sep;30(3):255-9. doi: 10.1097/WNO.0b013e3181dee96c.

DOI:10.1097/WNO.0b013e3181dee96c
PMID:20548243
Abstract

Bing-Neel syndrome (BNS) is defined as intracranial involvement of Waldenström macroglobulinemia (WM). Few cases of orbital involvement have been reported. A 51-year-old man with a history of WM developed bilateral orbitopathy and optic neuropathy. Orbital biopsy, cerebrospinal fluid studies, and neuroimaging confirmed the diagnosis of BNS involving the orbital soft tissues, optic nerves, meninges, and cauda equina. The neuro-ophthalmic manifestations resolved after parenteral and intrathecal chemotherapy in addition to autologous stem cell transplantation. The rare neuro-ophthalmic manifestations of BNS may require a multifaceted approach to therapy.

摘要

宾-尼尔综合征(BNS)被定义为瓦尔登斯特伦巨球蛋白血症(WM)的颅内受累。已有少数眼眶受累的病例报道。一名 51 岁男性,WM 病史,出现双侧眼眶病和视神经病变。眼眶活检、脑脊液研究和神经影像学检查证实了 BNS 的诊断,涉及眼眶软组织、视神经、脑膜和马尾。在进行全身和鞘内化疗以及自体干细胞移植后,神经眼科表现得到缓解。BNS 罕见的神经眼科表现可能需要多方面的治疗方法。

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