Susac syndrome

Epidemiology

 * Prevalence: 0.148 per 100,000 people
 * Annual incidence: 0.030-0.088 per 100,000 people
 * Gender: female 3-4x more often than males
 * Median age: 30-35 years

Signs and symptoms

 * Neurological findings (include MRI white matter T2/FLAIR hyperintensities)
 * Eye findings
 * Branch retinal artery occlusion
 * OR arterial wall hyperfluorescence in retinal fluorescein angiography)
 * OR signs of retinal branch ischemia on fundoscopy or optical coherence tomography
 * Ear findings
 * Tinnitus
 * OR hearing loss
 * OR vertigo

Treatment
Generally treatment is continued for at least 2 years.
 * Corticosteroids
 * IVIG
 * Mycophenolate mofetil
 * Additional immunotherapies
 * Tacrolimus
 * Cyclophosphamide
 * Rituximab