Microangiopathy of the brain and retina with hearing loss in a 50 year old woman: extending the spectrum of Susac’s syndrome
- aDepartment of Neurology, Norfolk and Norwich Health Care Trust, Brunswick Road, Norwich, Norfolk NR 1 3SR, UK, bDepartment of Pathology, cDepartment of Opthalmology, dDepartment of Neurology, Addenbrooke’s Hospital, Cambridge CB 2 2QQ, UK
- Dr Roger Barker, Department of Neurology, Addenbrooke’s Hospital, Cambridge CB 2 2QQ, UK. Telephone 0044 01223 245151; fax 0044 01223 33694.
- Received 15 April 1998
- Revised 26 June 1998
- Accepted 29 June 1998
Abstract
A 50 year old woman presented with a subacute onset of vertigo and diplopia followed by an encephalopathy with confusion, spasticity, ataxia, myoclonus, and multiple branch retinal arteriolar occlusions and unilateral sensorineural deafness. Brain biopsy confirmed multiple microinfarcts with no vasculitis. After the procedure she had a right iliofemoral deep vein thrombosis and was found to be heterozygous for the factor V Leiden mutation. She was 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 be associated with abnormalities of coagulation.
