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首页> 外文期刊>European journal of neurology: the official journal of the European Federation of Neurological Societies >Primary angiitis of the central nervous system: report of eight cases from Southern China.
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Primary angiitis of the central nervous system: report of eight cases from Southern China.

机译：中枢神经系统原发性血管炎：来自中国南方的八例报告。

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摘要

BACKGROUND AND PURPOSE: This study is to report the clinical, neuroimaging and pathological characteristics of patients with primary angiitis of the central nervous system (PACNS) from Southern China. METHODS: Eight patients with PACNS admitted between August 1995 and April 2006 were retrospectively studied. Records of clinical features, neuroimaging, brain biopsy and therapy were analyzed. RESULTS: Primary angiitis of the central nervous system occurred predominantly in youth and middle-aged adults. Headache, hemiplegia and speech disturbance were the most predominant manifestations. The cerebrospinal fluid (CSF) was slightly abnormal in only one case, MRI was abnormal in seven, magnetic resonance angiography in seven, diffusion-weighted imaging in five and digital subtraction angiography in four. Brain biopsy in four cases revealed lymphocytic angiitis. All cases had good outcome with the treatment of single corticoid or cyclophosphamide. CONCLUSION: We report eight cases of PACNS from Southern China associated with neurological and neuroimaging abnormalities; these patients presented a mild to moderate inflammatory disease that was correlated with few CSF abnormalities and good response to single steroid or cyclophosphamide treatment without relapses. Although brain biopsy represents the gold standard for diagnosis of PACNS, considering the difficulty and challenge of identification of this disease, combination with other examinations might be necessary to arrive at an early and definitive diagnosis.
机译：背景与目的：本研究旨在报道华南地区原发性中枢神经系统血管炎（PACNS）患者的临床，神经影像学和病理学特征。方法：回顾性研究1995年8月至2006年4月期间收治的8例PACNS患者。分析了临床特征，神经影像学，脑活检和治疗的记录。结果：中枢神经系统原发性血管炎主要发生在青年和中年成年人中。头痛，半身不遂和言语障碍是最主要的表现。脑脊液（CSF）仅有1例轻微异常，MRI异常7例，磁共振血管造影7例，弥散加权成像5例，数字减影血管造影4例。 4例脑活检显示淋巴细胞性血管炎。所有患者使用单一皮质类固醇激素或环磷酰胺治疗均取得良好的效果。结论：我们报告了8例华南地区PACNS患者的神经系统和神经影像异常。这些患者表现出轻度至中度的炎症性疾病，与少数CSF异常有关，并且对单一类固醇或环磷酰胺的治疗反应良好，且无复发。尽管脑活检是诊断PACNS的金标准，但考虑到识别这种疾病的困难和挑战，为早日确定性诊断，可能需要与其他检查相结合。

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