Susac syndrome: a vasospastic disorder?

PURPOSE: The Susac syndrome is a microangiopathy that leads to visual symptoms, hearing loss and neurological symptoms. CASE REPORT: We report on a young woman suffering from this syndrome who also presented the following signs and symptoms typical of a vasospastic syndrome; 1) a history of cold hands, low blood pressure and migraine; 2) a typical alteration of conjunctival vessels; 3) prolonged flow arrest time after cooling in nailfold capillaromicroscopy; 4) increased resistivity in the orbital vessels measured by color Doppler imaging; and 5) an increased plasma level of endothelin-1.
CONCLUSIONS: We postulate that the Susac syndrome is a manifestation of the vasospastic syndrome.