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[一例伴有短暂性白质脑病和多发性脑出血的髓过氧化物酶-抗中性粒细胞胞浆抗体相关性血管炎]

[A case of MPO-ANCA related vasculitis with transient leukoencephalopathy and multiple cerebral hemorrhage].

作者信息

Honda H, Hasegawa T, Morokawa N, Kato N, Inoue K

机构信息

Department of Neurology, Jikei University School of Medicine.

出版信息

Rinsho Shinkeigaku. 1996 Sep;36(9):1089-94.

PMID:8976134
Abstract

Recently, it has become clear that anti-neutrophil cytoplasmic autoantibody (ANCA) can be detected in patients with idiopathic necrotizing and crescentic glomerulonephritis or necrotizing angitis, and can be used as a serological marker for vasculitic syndrome. Described is a 56-year-old woman who had peripheral neuropathy, intestinal hemorrhage, generalized tonic-clonic convulsions, during the course of a rapidly progressive glomerulonephritis syndrome due to myeloperoxidase (MPO)-ANCA related vasculitis. The cranial MRI showed transient leukoencephalopathy in the parieto-occipital lobes bilaterally. The following MRI revealed multiple cortical and subcortical hemorrhage in the left occipital and right temporal lobe. The neurologic examination showed a slight left hemiparesis and a right homonymous hemianopsia, but, she experienced no subjective symptoms. In the case of MPO-ANCA related vasculitis, there is the possibility of intracranial disorders seen as this case. Thus, MPO-ANCA in related vasculitis should be always considered as a differential diagnosis in cases of transient localized leukoencephalopathy or cortical-subcortical cerebral hemorrhage.

摘要

最近,已明确在特发性坏死性新月体性肾小球肾炎或坏死性血管炎患者中可检测到抗中性粒细胞胞浆自身抗体(ANCA),且其可作为血管炎综合征的血清学标志物。本文描述了一名56岁女性,在因髓过氧化物酶(MPO)-ANCA相关血管炎导致的快速进展性肾小球肾炎综合征病程中,出现了周围神经病变、肠道出血、全身强直阵挛性惊厥。头颅MRI显示双侧顶枕叶短暂性白质脑病。随后的MRI显示左侧枕叶和右侧颞叶多发皮质及皮质下出血。神经系统检查显示轻度左侧偏瘫和右侧同向性偏盲,但她无主观症状。在MPO-ANCA相关血管炎的情况下,存在像该病例中所见的颅内病变的可能性。因此,在出现短暂性局限性白质脑病或皮质-皮质下脑出血的病例中,应始终将MPO-ANCA相关血管炎视为鉴别诊断的考虑因素。

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