Intracerebral hemorrhage associated with Sneddon's syndrome: is ischemia-related angiogenesis the cause? Case report and review of the literature

Abstract

Sneddon's syndrome is characterized by livedo reticularis and multiple ischemic infarcts often associated with antiphospholipid antibodies. Intracerebral hemorrhage (ICH) is unusual in Sneddon's syndrome and has not been reported as the presenting complaint. We report a 38-year-old woman with a history of two miscarriages, Raynaud's phenomenon and livedo reticularis who presented acutely with ICH. Angiography showed prominent leptomeningeal and transdural anastomoses (pseudoangiomatosis). Anticardiolipin antibodies were positive. A right frontal brain biopsy failed to reveal vasculitis and a skin biopsy was nonspecific. MRI showed residual intracerebral hemorrhage (ICH), diffuse atrophy, multiple small white matter infarcts and leptomeningeal enhancement. This is the first report of Sneddon's syndrome presenting with an ICH. It shares features with the Divry-van Bogaert syndrome. We discuss the cause of the pseudoangiomatosis pattern and its role in the genesis of the hemorrhage and suggest that cerebral angiography should be done in every patient with Sneddon's syndrome, as it could impact therapy.

The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa

“…Aquino Gondim believes that haemorrhage might be due to rupture of an abnormal vessel, similar to the mechanism described in moyamoya [2], particularly in cases with intracerebral haemorrhages. It can be hypothesized that this "pseudoangiomatosis" is a marker of high risk for haemorrhage in SS and thus proposed that cerebral angiography should be performed in all patients with SS.…”

“…Many authors described no abnormalities and no evidence of vasculitis in cerebral angiograms. Others have reported a variety of findings including irregular vessel calibres, medium and small vessel stenosis, occlusion or stenosis of major cerebral vessels, transdural anastomoses, large networks of fine collateral vessels and granulomatous leptomeningeal infiltration [2,33,93]. In 2003, Aquino Gondim et al reported a case of lobar intracerebral haemorrhage with an angiographic pattern of "pseudoangiomatosis" as the initial manifestation of SS [2].…”

“…Angiography shows prominent leptomeningeal and transdural anastomoses. This large network of collateral vessels has been called "angiomatosis".However,it can be argued that this is a misnomer because there is no evidence of a true neoplastic vascular proliferation [2]. Some groups have referred to it as a "pseudoangiomatosis", because it is a vascular dilatation with transdural anastomoses [2,76].…”

“…Historically the distinguishing features between Divry-van Bogaert syndrome and SS have been the presence of cerebromeningeal angiomatosis and the familiar presentation of Divry-van Bogaert syndrome [94,96], although there have also been several reports of vascular dilatation with leptomeningeal and transdural anastomoses in SS [2].Adding to the confusion, an autosomal dominant and recessive inheritance in SS has been reported [94].…”

“…It has been argued that the Divry-van Bogaert syndrome merely represents an infanto-juvenile version of SS [25]. Other authors still differentiate between these two entities because of the presence of anticardiolipin antibodies in SS and the difference in incidence between the sexes [2,94], although this distinction seems to be implausible.…”