Muerza F M, González G, Ortiz E, Saracibar N
Sección de Neurología, Hospital Txagorritxu, Vitoria, Alava, España.
Rev Neurol. 1998 Jul;27(155):74-6.
Sneddon syndrome is the association of livedo retricularis and cerebrovascular lesions. It is a vasculopathy of vessels of small and medium calibre which affects young people and is of unknown origin. It is often associated with arterial hypertension and repeated ischemic cerebrovascular accidents which lead to intellectual deterioration.
We describe a 49 year old man who, from the age of 23, had had repeated transient or established episodes compatible with ischemic cerebrovascular accidents. These were followed by various neurological sequelae and by intellectual deterioration. Finally he died of a cerebral hemorrhage whilst on anticoagulant treatment with Synthron. We show the neuroimaging findings (CT, MR and angiography) and the neuropathological studies (cerebral hemorrhage, fibrosis of the intima of the vessels of the circle of Willis, absence of inflammation, bilateral unsymmetrical leukoencephalopathy of the cerebral hemispheres and multiple cortical infarcts).
We wish to draw attention to the occurrence of this complication, its possible relationship to the vasculopathy mentioned, bursting of one of the arteries of the revascularization complex, arterial hypertension or anticoagulant treatment, and also to add one more case to the few described as having hemorrhage and neuropathology in Sneddon syndrome.
斯内登综合征是网状青斑与脑血管病变的关联。它是一种影响年轻人的中小口径血管的血管病,病因不明。它常与动脉高血压及反复发生的缺血性脑血管意外相关,后者会导致智力衰退。
我们描述一名49岁男性,自23岁起就反复出现与缺血性脑血管意外相符的短暂或持续性发作。随后出现各种神经后遗症及智力衰退。最终,他在接受辛酮抗凝治疗期间死于脑出血。我们展示了神经影像学检查结果(CT、磁共振成像和血管造影)及神经病理学研究结果(脑出血、 Willis 环血管内膜纤维化、无炎症、双侧不对称性大脑半球白质脑病及多发性皮质梗死)。
我们希望提请注意这种并发症的发生、它与上述血管病的可能关系、血管重建复合体的一条动脉破裂、动脉高血压或抗凝治疗,并且在少数已描述的斯内登综合征伴有出血和神经病理学改变的病例中再增加一例。