Branch retinal artery occlusions as the presenting feature of primary central nervous system vasculitis

J.O. Susac¹, L.H. Calabrese², E. Baylin³, R.A. Prayson⁴, N.E. Medeiros⁵, R.P. Hull⁶, J.P. Tucker⁷

¹Neurology & Neurosurgery, Winter Haven, Florida; ²Division of Rheumatology, ³Division of Ophthalmology, and ⁴Division of Neuropathology, Cleveland Clinic, Cleveland, Ohio; ⁵Retinal Specialists of North Alabama, Huntsville; ⁶Neurology, Huntsville, Alabama; ⁷Riverbend Family Medicine, Scottsboro, Alabama, USA.

ABSTRACT
A 39-year-old woman presented with multiple branch retinal artery occlusions almost three years before developing a mass lesion containing calcium in the left frontal lobe. Brain biopsy revealed a small vessel vasculitis and ischemic necrosis of brain with dystrophic calcification. We believe this to be the first case of primary CNS vasculitis with branch retinal artery occlusions and brain calcification.

Key words
Primary CNS vasculitis, Susac's syndrome, branch retinal artery occlusions, MRI, brain calcification, CT.

Please address correspondence to J.O. Susac, Neurology and Neurosurgery Associates, 50 2nd Street, S.E. Winter Haven, Florida 33880, USA.
E-mail: jsusac@neurohaven.com

Clin Exp Rheumatol 2004; 22 (Suppl. 36): S70-S74.
© Copyright Clinical and Experimental Rheumatology 2004.