与抗磷脂综合征相关的结节性多动脉炎的神经学表现。

Annales de medecine interne Pub Date : 2003-11-01
Laurence Valeyrie, Nicolas Bachot, Jean-Claude Roujeau, Jérôme Authier, Romain Gherardi, Hassan Hosseini
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引用次数: 0

摘要

抗磷脂综合征(APS)和血管炎之间的联系是罕见的,并继续引起极大的兴趣。我们报告一例神经学表现为结节性多动脉炎(PAN)并并发APS。肢体肌电图和神经肌肉活检显示中等动脉闭塞和坏死后的轴突多神经病变,初步提示PAN。此血管炎在内脏选择性动脉造影中被证实,伴有许多动脉瘤。脑MRI显示额顶区皮层和皮层下多发信号,与APS缺血性微血管病变相对应。抗心脂IgG的存在、皮肤坏死性病变的既往病史和中央视网膜闭塞证实了这种关联。脉搏静脉注射环磷酰胺、口服强的松龙和治愈性抗凝治疗稳定8个月。
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Neurological manifestations of polyarteritis nodosa associated with the antiphospholipid syndrome.

The association between antiphospholipid syndrome (APS) and vasculitis is rare and continues to evoke great interest. We report a case with neurological manifestations of polyarteritis nodosa (PAN) and concurrent APS. Electromyography and neuromuscular biopsy of the limb showed an axonal polyneuropathy following obliteration and necrosis of medium sized arteries, initially suggesting PAN. This vasculitis was confirmed on visceral selective arteriography, with the presence of numerous aneurysms. Cerebral MRI revealed multiple cortical and subcortical signals in the fronto-parietal areas, corresponding to ischemic microvascular lesions of APS. This association was confirmed by the presence IgG anticardiolipid, the past medical history of skin necrotic lesions and central retinal obliteration. Pulse intravenous cyclophosphamide, oral prednisolone and curative anticoagulation led to stabilization for 8 months.

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