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Abstract：
BACKGROUND: We present a rare case of NeuroBehcet\'s-related intracranial hypertension without cerebral venous thrombosis (NBrIHwCVT), occurring as the first presentation of NeuroBehcet\'s. In addition, we describe the novel use of subcutaneous tocilizumab for this indication. This is followed by a review of the literature on this topic.
METHODS: The patient was a 28-year-old lady of Southern Chinese origin with a known history of Behcet\'s disease with oral ulcers and ocular findings for which she was on mycophenolate mofetil and adalimumab. She presented with a headache and bilateral disc swelling associated with an intracranial pressure (ICP) of > 40cmH20. There were no structural lesions or cerebral venous thrombosis (CVT) on imaging. Initial lumbar puncture had raised leucocytes and protein. We discuss diagnostic challenges given persistently elevated ICP despite subsequent non-inflammatory cerebrospinal fluid (CSF) profiles and non-response to acetazolamide. She eventually showed a response to immunosuppressant therapy in the form of pulsed methylprednisolone, cyclophosphamide and subsequently subcutaneous tocilizumab, supporting the diagnosis of NBrIHwCVT. Complete normalization of ICP remains challenging. Her disease course was severe, unusual for her ethnicity.
METHODS: We identified 34 patients (including ours) from 14 publications. We found that the majority of NBrIHwCVT patients were young (average age of 34 years), with a slight female preponderance. Of the 17 cases in the literature with available data on CSF profile, none had raised leucocytes whilst one patient had elevated protein. Patients were generally treated with steroids and occasionally azathioprine, in line with the suspected autoimmune pathophysiology. Of 22 patients with data on outcome, six (27%) were noted to have recurrence of symptoms generally occurring a few months later.
CONCLUSIONS: As demonstrated by this case, NBrIHwCVT can present with BD with raised ICP even if there is no prior history of NB, central Asian ethnicity, cerebral venous thrombosis or features of inflammation on the CSF. We demonstrated how novel use of Tocilizumab may have a role in the management of NBrIHwCVT. Based on our literature review, patients were more likely to be young, female, display a non-inflammatory CSF picture, be treated with steroids and harbour a possibility of recurrence.
METHODS: The patient was a 28-year-old lady of Southern Chinese origin with a known history of Behcet\'s disease with oral ulcers and ocular findings for which she was on mycophenolate mofetil and adalimumab. She presented with a headache and bilateral disc swelling associated with an intracranial pressure (ICP) of > 40cmH20. There were no structural lesions or cerebral venous thrombosis (CVT) on imaging. Initial lumbar puncture had raised leucocytes and protein. We discuss diagnostic challenges given persistently elevated ICP despite subsequent non-inflammatory cerebrospinal fluid (CSF) profiles and non-response to acetazolamide. She eventually showed a response to immunosuppressant therapy in the form of pulsed methylprednisolone, cyclophosphamide and subsequently subcutaneous tocilizumab, supporting the diagnosis of NBrIHwCVT. Complete normalization of ICP remains challenging. Her disease course was severe, unusual for her ethnicity.
METHODS: We identified 34 patients (including ours) from 14 publications. We found that the majority of NBrIHwCVT patients were young (average age of 34 years), with a slight female preponderance. Of the 17 cases in the literature with available data on CSF profile, none had raised leucocytes whilst one patient had elevated protein. Patients were generally treated with steroids and occasionally azathioprine, in line with the suspected autoimmune pathophysiology. Of 22 patients with data on outcome, six (27%) were noted to have recurrence of symptoms generally occurring a few months later.
CONCLUSIONS: As demonstrated by this case, NBrIHwCVT can present with BD with raised ICP even if there is no prior history of NB, central Asian ethnicity, cerebral venous thrombosis or features of inflammation on the CSF. We demonstrated how novel use of Tocilizumab may have a role in the management of NBrIHwCVT. Based on our literature review, patients were more likely to be young, female, display a non-inflammatory CSF picture, be treated with steroids and harbour a possibility of recurrence.
摘要：
背景：我们介绍了一例罕见的NeuroBehcet相关性颅内高压而无脑静脉血栓形成（NBrIHwCVT），作为NeuroBehcet的第一次出现。此外,我们描述了皮下托珠单抗用于该适应症的新用途。接下来是对有关该主题的文献的回顾。
方法：患者是一名28岁的中国南方女性，有已知的Behcet病的口腔溃疡和眼部发现，她正在服用吗替麦考酚酯和阿达木单抗。患者出现头痛和双侧椎间盘肿胀，颅内压（ICP）>40cmH20。影像学上无结构性病变或脑静脉血栓形成（CVT）。最初的腰椎穿刺增加了白细胞和蛋白质。我们讨论了尽管随后的非炎症性脑脊液（CSF）谱和对乙酰唑胺无反应，但ICP持续升高的诊断挑战。她最终表现出对脉冲甲基强的松龙形式的免疫抑制剂治疗的反应，环磷酰胺和随后皮下托珠单抗，支持NBrIHwCVT的诊断。ICP的完全正常化仍然具有挑战性。她的病情很严重,不寻常的她的种族。
方法：我们从14篇出版物中确定了34名患者（包括我们的患者）。我们发现大多数NBrIHwCVT患者都是年轻人（平均年龄34岁），有轻微的女性优势。在文献中的17例病例中，有关于CSF概况的可用数据，没有一个患者的白细胞升高，而一名患者的蛋白质升高。患者通常使用类固醇治疗，偶尔使用硫唑嘌呤，符合疑似自身免疫病理生理学。在有结果数据的22名患者中，6例(27%)的患者发现症状通常在几个月后复发.
结论：如案例所示，NBrIHwCVT可以与BD一起出现升高的ICP，即使没有先前的NB病史，中亚种族，脑静脉血栓形成或CSF上的炎症特征。我们证明了Tocilizumab的新用途如何在NBrIHwCVT的管理中发挥作用。根据我们的文献综述，患者更有可能年轻，女性,显示非炎性CSF图片，用类固醇治疗，并有复发的可能性。
方法：患者是一名28岁的中国南方女性，有已知的Behcet病的口腔溃疡和眼部发现，她正在服用吗替麦考酚酯和阿达木单抗。患者出现头痛和双侧椎间盘肿胀，颅内压（ICP）>40cmH20。影像学上无结构性病变或脑静脉血栓形成（CVT）。最初的腰椎穿刺增加了白细胞和蛋白质。我们讨论了尽管随后的非炎症性脑脊液（CSF）谱和对乙酰唑胺无反应，但ICP持续升高的诊断挑战。她最终表现出对脉冲甲基强的松龙形式的免疫抑制剂治疗的反应，环磷酰胺和随后皮下托珠单抗，支持NBrIHwCVT的诊断。ICP的完全正常化仍然具有挑战性。她的病情很严重,不寻常的她的种族。
方法：我们从14篇出版物中确定了34名患者（包括我们的患者）。我们发现大多数NBrIHwCVT患者都是年轻人（平均年龄34岁），有轻微的女性优势。在文献中的17例病例中，有关于CSF概况的可用数据，没有一个患者的白细胞升高，而一名患者的蛋白质升高。患者通常使用类固醇治疗，偶尔使用硫唑嘌呤，符合疑似自身免疫病理生理学。在有结果数据的22名患者中，6例(27%)的患者发现症状通常在几个月后复发.
结论：如案例所示，NBrIHwCVT可以与BD一起出现升高的ICP，即使没有先前的NB病史，中亚种族，脑静脉血栓形成或CSF上的炎症特征。我们证明了Tocilizumab的新用途如何在NBrIHwCVT的管理中发挥作用。根据我们的文献综述，患者更有可能年轻，女性,显示非炎性CSF图片，用类固醇治疗，并有复发的可能性。
