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[一例未行活检的炎症性淀粉样血管病应用皮质类固醇治疗的反应]

[Response to treatment with corticoids in a case of inflammatory amyloid angiopathy without performing a biopsy].

作者信息

de la Riva Patricia, Moreno Fermín, Carrera Nieves, Barandiarán Myriam, Arruti Maialen, Martí-Massó José F

机构信息

Servicio de Neurología, Hospital Donostia, San Sebastián, Guipúzcoa, España.

出版信息

Rev Neurol. 2012 Oct 1;55(7):408-12.

Abstract

INTRODUCTION

Inflammatory amyloid angiopathy (IAA) is an infrequent presenting symptom of the recently recognised cerebral amyloid angiopathy and its definitive diagnosis is reached by means of pathological analyses.

AIM

We report the case of a male patient with IAA and good clinical, neuropsychological and neuroimaging response to treatment with corticoids; a biopsy of brain tissue was not considered necessary.

CASE REPORT

The patient, 68 years old and diagnosed with Alzheimer's disease, suffered from generalised seizures followed by a language disorder and hemiparesis of the right-hand side. A magnetic resonance imaging scan showed a lesion displaying infiltrating behaviour in the left hemisphere and multiple instances of microbleeding. Clinical and radiological features suggested IAA and treatment was established with corticoids. Neuroimaging and neuropsychological tests revealed a notable improvement at 30 days after beginning treatment with immunosuppressants. The genotype was ApoE e4/e4. The need to perform a biopsy of brain tissue was ruled out.

CONCLUSIONS

The case described here suggests that, in individualised cases with clinical and radiological features that are characteristic of IAA, it may be possible to establish an empirical treatment with corticoids with a probability diagnosis and perform a biopsy of brain tissue in the event of a lack of response to treatment.

摘要

引言

炎症性淀粉样血管病(IAA)是最近认识到的脑淀粉样血管病的一种不常见的表现症状,其确诊需通过病理分析。

目的

我们报告一例患有IAA的男性患者,其在接受皮质类固醇治疗后临床、神经心理学和神经影像学方面均有良好反应;未考虑进行脑组织活检。

病例报告

该患者68岁,诊断为阿尔茨海默病,出现全身性癫痫发作,随后出现语言障碍和右侧偏瘫。磁共振成像扫描显示左侧半球有一个表现为浸润性的病变以及多处微出血。临床和放射学特征提示为IAA,并开始用皮质类固醇进行治疗。神经影像学和神经心理学测试显示,在开始使用免疫抑制剂治疗30天后有显著改善。基因型为ApoE e4/e4。排除了进行脑组织活检的必要性。

结论

此处描述的病例表明,在具有IAA特征性临床和放射学表现的个体化病例中,有可能在可能的诊断下用皮质类固醇进行经验性治疗,并且在治疗无反应的情况下进行脑组织活检。

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