Microangiopathy of the brain, retina, and cochlea (susac syndrome) A report of five cases and a review of the literature

The Emerging Link Between Autoimmune Disorders and Neuropsychiatric Disease

“…Susac’s syndrome is thought to be a brain microangiopathy, with pathologic findings similar to those of autoimmune antiendothelial cell antibody syndromes. 53 Patients respond well if the syndrome is identified early and treated with immunosuppression, 54,55 further supporting the notion that Susac’s syndrome is an autoimmune disorder.…”

“…Susac’s syndrome, first described in 1979, consists of the triad of branch retinal arterial occlusions, hearing loss, and acute encephalopathy (Table 2). 52–54 Neuropsychiatric symptoms may be less global, however, and include personality changes, paranoia, and affective dysregulation early in the course, with memory abnormalities and increasing confusion as the syndrome progresses. 54,55 Focal neurologic findings and headache are also commonly observed.…”

“…52–54 Neuropsychiatric symptoms may be less global, however, and include personality changes, paranoia, and affective dysregulation early in the course, with memory abnormalities and increasing confusion as the syndrome progresses. 54,55 Focal neurologic findings and headache are also commonly observed. 54 Susac’s syndrome usually affects young women, and a viral prodrome can precede other symptoms.…”

“…54,55 Focal neurologic findings and headache are also commonly observed. 54 Susac’s syndrome usually affects young women, and a viral prodrome can precede other symptoms. MRI shows disseminated lesions in white and gray matter, with a predilection for corpus callosum involvement.…”

“…EEG often reveals diffuse slowing, whereas CSF only reliably shows elevated protein (no definitive autoantibody has been identified as causative). 54,56 Fluorescein angiography and audiometry are normally part of the diagnostic process, as well. Susac’s syndrome is thought to be a brain microangiopathy, with pathologic findings similar to those of autoimmune antiendothelial cell antibody syndromes.…”