Serial retinal fluorescein angiography and immune therapy in Susac's syndrome.
We report a patient with Susac's disease presenting classically in a young female with an encephalopathy and visual disturbance with later deafness and tinnitus. Her encephalopathy settled, but subsequent serial fluorescein angiograms allowed sensitive monitoring of continuing sub-clinical disease activity, and provide evidence of a clear therapeutic response to immune suppression with tacrolimus (but not steroids alone)--and of a lack of efficacy of nimodipine and aspirin. We believe this single case study has both pathogenetic and useful practical implications: the apparently favourable response to immunosuppression lends support to the hypothesis that Susac's Syndrome is an immune-mediated disease; while the presence during symptomatic clinical remission of sporadic, multi-focal episodes of hyper-fluorescence, suggestive of breakthrough vasculopathy despite treatment, underlines the fact that the natural history of this rare condition is still not fully understood. Fluorescein angiography is proposed as a sensitive and important approach to the monitoring of sub-clinical disease activity, and so optimising immune suppressive treatment.
Mallam
Beth
B
Neurology Department, University of Bristol Institute of Clinical Neurosciences, Frenchay Hospital, Bristol BS16 1LE, United Kingdom.
Damato
Erika M
EM
Scolding
Neil J
NJ
Bailey
Clare
C
D000328:Adult; D000792:Angiography; D001921:Brain / Q000473:pathology; D003072:Cognition Disorders / Q000473:pathology* / Q000628:therapy*; D018450:Disease Progression; D005260:Female; D019793:Fluorescein; D006261:Headache / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D007167:Immunotherapy; D008279:Magnetic Resonance Imaging; D012160:Retina / Q000473:pathology; D013577:Syndrome; D014012:Tinnitus / Q000473:pathology* / Q000628:therapy*; D016896:Treatment Outcome; D044465:White People
D019793:Fluorescein