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以肿瘤样病变为表现的神经白塞病对利妥昔单抗治疗有反应。

Neuro-Behçet's disease presenting with tumour-like lesions and responding to rituximab.

机构信息

Waikato DHB, Hamilton 3204, New Zealand.

Sydney Adventist Hospital Clinic, Wahroonga, NSW 2076, Australia.

出版信息

J Clin Neurosci. 2016 Oct;32:139-41. doi: 10.1016/j.jocn.2016.03.020. Epub 2016 Jun 16.

DOI:10.1016/j.jocn.2016.03.020
PMID:27320374
Abstract

We describe a patient with neuro-Behçets disease (NBD) that presented with symptoms of raised intracranial pressure including papilloedema. MRI revealed tumour-like lesions which, on biopsy, confirmed an active vasculitis. Treatment was commenced with prednisone and cyclophosphamide which proved unsuccessful with enlargement of the cerebral mass lesions. Infliximab and mycophenolate were trialled also without benefit. The patient required ventriculoperitoneal shunts to relieve the symptoms of hydrocephalus. Rituximab was then commenced with significant symptomatic and imaging improvement. The case is unique, in our experience, in the need for shunting to relieve the symptoms of hydrocephalus related to vasculitis.

摘要

我们描述了一位患有神经白塞病(NBD)的患者,其表现为颅内压升高的症状,包括视乳头水肿。MRI 显示出肿瘤样病变,活检证实为活跃的血管炎。治疗开始使用泼尼松和环磷酰胺,但脑肿块病变增大,治疗无效。使用英夫利昔单抗和霉酚酸酯治疗也没有效果。患者需要脑室-腹腔分流术来缓解脑积水的症状。然后开始使用利妥昔单抗,症状和影像学均有显著改善。据我们所知,该病例的独特之处在于需要分流术来缓解与血管炎相关的脑积水症状。

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