Microangiopathy of the brain and retina with hearing loss in a 50 year oldwoman: extending the spectrum of Susac's syndrome

A 50 year old woman presented with a subacute onset of vertigo and diplopia followed by an encephalopathy with confusion, spasticity, ataxia, myoclonu s, and multiple branch retinal arteriolar occlusions and unilateral sensori neural deafness. Brain biopsy confirmed multiple microinfarcts with no vasc ulitis. After the procedure she had a right iliofemoral deep vein thrombosi s and was found to be heterozygous for the factor V Leiden mutation. She wa s treated with anticoagulants and made a marked recovery with no relapses 6 months after presentation. This case extends the age range at which Susac' s syndrome can present, and raises the possibility that the condition may b e associated with abnormalities of coagulation.