Susac syndrome: a vasospastic disorder?

PURPOSE. The Susac syndrome is a microangiopathy that leads to visual sympt oms, hearing loss and neurological symptoms. CASE REPORT. We report on a young woman suffering from this syndrome who al so presented the following signs and symptoms typical of a vasospastic synd rome; I) a history of cold hands, low blood pressure and migraine; 2) a typ ical alteration of conjunctival vessels; 3) prolonged flow arrest time afte r cooling in nailfold capillaromicroscopy; 4) increased resistivity in the orbital vessels measured by color Doppler imaging; and 5) an increased plas ma level of endothelin-l. CONCLUSIONS. We postulate that the Susac syndrome is a manifestation of the vasospastic syndrome.