Hemorragia cerebral en el síndrome de Sneddon

Abstract

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.