Lisa Eilenberg Søgaard, Zsolt Illes, Nina Nguyen & Keld-Erik Byg:
Cognitive dysfunction, loss of visual acuity and hearing can be symptoms of Susac syndrome
Ugeskr Læger 2019;181:V06180437
This is a case report of a 56-year-old male patient with Susac syndrome. The syndrome is a rare immune-mediated, ischaemic, occlusive microvascular endotheliopathy affecting the brain, retina and inner ear. Diagnosis and treatment are challenging, since the presentation shows great variability. Brain MRI, fundoscopy and audiometry enable the diagnosis. Early treatment with immunosuppressive medication is crucial and can reduce complications like dementia, loss of visual acuity and hearing. The treatment is empirical and based on the fact, that the histopathology of the syndrome is similar to juvenile dermatomyositis.