Microangiopathy of the brain and retina

Abstract

Two women (26 and 40 yr old) developed an unusual microangiopathy that affected the brain and retina. Psychiatric symptoms initially overshadowed the subacute features of the progressive neurologic disorder. Ophthalmoscopic findings of multifocal branch retinal artery occlusions provided clinical evidence of vasculopathy. Laboratory data did not reveal evidence of the known vasculitides, including systemic lupus erythematosus (SLE) and syphilis. Cerebral angiography suggested vasculitis in the younger patient. Brain biopsy in the older patient (after 3 mo. of steroid therapy) revealed a sclerosis of the small pial and cortical vessels that was consistent with a healed angiitis. Both patients seemed to respond to steroid therapy.