title: Susac's syndrome: an immune mediated endotheliopathy laden with challenges and controversies.
abstract: Susac's syndrome is a microangiopathy of the retina, inner ear and brain manifesting as a triad of encephalopathy, hearing loss and branch retinal artery occlusion. The pathological mechanism is thought to be an immune-mediated small vessel vasculitis with some pathophysiological similarity to dermatomyositis. Awareness and early recognition of this syndrome is important as early treatment with immunosuppression can minimise cognitive, audiological and visual sequelae. We report a case of a 33-year-old woman who presented with the characteristic syndrome. She was treated with immunomodulatory therapy and remains well 2 years postpresentation with no new events.
authors: Department of Neurology, Barwon Neurology, Geelong, Victoria, Australia. dr.malhotra@gmail.com
authors: Abhishek
authors: A
authors: Malhotra
authors: Elizabeth
authors: E
authors: Reyneke
authors: Merrilee
authors: M
authors: Needham
mesh_terms: D000328:Adult; D001301:Audiometry, Pure-Tone; D003937:Diagnosis, Differential; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D005451:Fluorescein Angiography; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D056784:Leukoencephalopathies / Q000175:diagnosis; D008775:Methylprednisolone / Q000627:therapeutic use; D009103:Multiple Sclerosis / Q000175:diagnosis*; D055955:Susac Syndrome / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D008775:Methylprednisolone
references: AJNR Am J Neuroradiol. 2004 Mar;25(3):351-2
references: 15037453
references: Curr Treat Options Neurol. 2008 Jan;10(1):67-74
references: 18325301
references: Rheum Dis Clin North Am. 2010 Feb;36(1):99-108, viii
references: 20202593
references: J Neurol Sci. 2010 Oct 15;297(1-2):71-3
references: 20723912