Susac et al’s syndrome (1979) consists of a triad of cerebral microangiopathy, retinal artery branch occlusions and hearing loss. The pathogenesis remains unknown. The severity of retinal vasculopathy is variable.
Case report: a 57-year-old Caucasian woman presented in 1999 with a severe and complete form of the disease. Fluorescein angiography and cerebral MRI confirmed the diagnosis. Severe unilateral visual loss occurred a month after the initiation of treatment with systemic corticoids. Fundoscopy showed a temporal retinal artery occlusion with major ischaemia. Cyclophosphamide and anticoagulants were added to the corticosteroids and total visual recovery was achieved within 2 weeks. There has been no relapse with a follow-up of 54 months.
Therapeutic management of Susac’s syndrome is still controversial. Major immunosuppressive therapy is mandatory where there is severe visual loss associated with central or branch retinal artery occlusion. The final prognosis seems favourable if prolonged symptomatic retinal ischaemia is absent.
© 2005 Elsevier Masson SAS. Tous droits réservés.