Intraventricular Hemorrhage as an Unusual Presenting Form of Sneddon Syndrome

BACKGROUND: Intraventricular hemorrhage, which has a poor prognosis, is an extremely rare presenting symptom of central nervous system vasculitis. Sneddon syndrome, which is a systemic vasculitic disease, generally presents with ischemic stroke and livedo reticularis. Intraventricular hemorrhage is extremely rare in Sneddon syndrome and has not been reported as the presenting complaint. METHODS: We report a 37-year-old woman who presented with acute intraventricular hemorrhage, and on further evaluation her condition was diagnosed as Sneddon syndrome. RESULTS: Patient underwent ventriculoperitoneal shunting operation for hydrocephalus and her condition markedly improved 6 months later; she was independent in her activities of daily living. CONCLUSIONS: In this report, we emphasize the importance of multisystemic evaluation of patients, especially those with obscure angiography findings.