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[脑血管炎]

[Cerebral vasculitides].

作者信息

de Boysson Hubert, Faivre Anthony, Pagnoux Christian

机构信息

CHU Côte-de-Nacre, université de Caen, pôle de médecine interne et d'immunologie clinique, avenue de la Côte-de-Nacre, 14033 Caen cedex 9, France.

出版信息

Presse Med. 2012 Nov;41(11):1071-83. doi: 10.1016/j.lpm.2011.12.009. Epub 2012 Feb 10.

Abstract

Cerebral vasculitides can be secondary to a broad variety of conditions such as systemic disease, infections, malignancies and toxic exposures, or can be isolated to the central nervous system. When cerebral vasculitis is suspected, the diagnostic work up must be exhaustive to rule out all these potential causes of secondary vasculitis. Most often, the diagnosis of cerebral vasculitis relies on the combination of clinical, biological and radiological findings. It is definite only when based on a positive leptomeningeal biopsy. This latter should be done when the diagnostic uncertainty remains high, especially in patients with underlying systemic disease and/or infrequent or rapidly-progressive symptoms. One can probably rule out the diagnostic of cerebral vasculitis in patients in whom both MRI and cerebrospinal fluid examination are normal. For secondary cerebral vasculitis, treatment depends on the identified underlying etiology. However, corticosteroids combined with immunosuppressive drugs are usually used. Actually, there is no consensual treatment for primary angiitis of the central nervous system. The prognosis is better when diagnosis is made early and treatment started promptly.

摘要

脑血管炎可继发于多种情况,如全身性疾病、感染、恶性肿瘤和接触毒物,也可局限于中枢神经系统。当怀疑有脑血管炎时,诊断检查必须全面,以排除所有这些继发性血管炎的潜在病因。大多数情况下,脑血管炎的诊断依赖于临床、生物学和影像学检查结果的综合判断。只有在软脑膜活检呈阳性时才能确诊。当诊断不确定性仍然很高时,尤其是在患有潜在全身性疾病和/或症状不常见或进展迅速的患者中,应进行软脑膜活检。对于MRI和脑脊液检查均正常的患者,可能可以排除脑血管炎的诊断。对于继发性脑血管炎,治疗取决于确定的潜在病因。然而,通常使用皮质类固醇联合免疫抑制药物。实际上,对于中枢神经系统原发性血管炎尚无共识性的治疗方法。早期诊断并及时开始治疗时,预后较好。

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