Clinical, neurovascular and neuropathological features in sneddon's syndrome

Abstract

Sneddon's syndrome (SS) is characterized by ischemic cerebrovascular episodes and livedo reticularis. It is more common in young women and can also be associated with valvulopathy, a history of spontaneous abortion, renal involvement and vascular dementia. We describe three cases of young women with this disease. The patients had repeated ischemic cerebral episodes, livedo reticularis and thrombocytopenia. CT and MRI showed strokes and cerebral atrophy. Autopsy in one of the patients revealed cerebral infarctions. Anticardiolipin antibodies were detected in two patients. Antiphospholipid antibodies may be found in some patients with ischemic cerebrovascular events and livedo reticularis. SS may thus be associated with antiphospholipid syndrome. We described three new cases of SS and discuss the pathophysiology of this disease.

Relation of Antiphospholipid Antibodies to Postmortem Brain Infarcts in Older People

“…These series describe thrombo-occlusive disease in the brain, small but also some large infarcts, and no inflammation or vasculitis. 7,23–26 One larger hospital-based autopsy series of 156 consecutive subjects with and without aPL, showed that aPL were common and associated with thrombi but not vasculitis. 8 …”

Strokes in Sneddon syndrome without antiphospholipid antibodies

“…Neuropathological data suggest a progressive cerebral vasculopathy of small-and mediumsize arteries, distinct from vasculitides, but only 10 neuropathological cases have been reported. [4][5][6][7][8][9][10][11][12][13] Cardiac abnormalities in SS without aPL, particularly valve disease, have been reported in case studies or small series as well, and this raises the question of their involvement in the genesis of IS. 14 The objective of this study was 2-fold: (1) to assess the neurological, cardiac, and systemic manifestations in SS without aPL and its prognosis and (2) to determine the pattern of IS and use it as a hallmark to better understand the pathophysiological mechanisms leading to a stroke.…”

Differenzialdiagnostik seltener Schlaganfallursachen

“…So zeigte sich in einer 6-jährigen prospektiven Observationsstudie bei 54 % der Patienten eine TIA, die jedoch ohne manifesten Infarkt einherging [119]. Nichtsdestotrotz sind diese beschrieben, wobei vor allem jüngere Frauen betroffen sind [120]. Therapeutisch empfiehlt sich eine orale Antikoagulation, eine immunsuppressive Therapie ist wahrscheinlich unwirksam.…”