Affiliation:
1. Central State Medical Academy of the Administrative Directorate of the President of the Russian Federation;
V.A. Nasonova Research Institute of Rheumatology;
Moscow Polytechnic University
Abstract
Susac syndrome (SS) or retino-cochleo-cerebral vasculopathy is an extremely rare, severe, and potentially disabling condition. Underlying occlusive microangiopathy in SS is clinically characterized by the triad of encephalopathy, sensorineural hearing loss and branch retinal arterial occlusion. SS therapy envisages simultaneous use of high doses of glucocorticoids, intravenous immunoglobulins, cyclophosphamide and rituximab (RTХ). This article presents a case of remitting-relapsing slow-progressive SS with typical clinical manifestations demonstrating successful treatment SS with RTХ monotherapy; it also discuss the focus of RTХ monotherapy should be targeted at SS cases with contraindications for glucocorticoids and cytostatics use, slow-progressive SS or at early stages.
Subject
Immunology,Immunology and Allergy,Rheumatology
Reference22 articles.
1. Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology. 1979;29(3):313-316. doi: 10.1212/wnl.29.3.313
2. Jarius S, Kleffner I, Dörr JM, Sastre-Garriga J, Illes Z, Eggenberger E, et al. Clinical, paraclinical and serological findings in Susac syndrome: An international multicenter study. J Neuroinflammation. 2014;11:46. doi: 10.1186/1742-2094-11-46
3. Nazari F, Azimi A, Abdi S. What is Susac syndrome? A brief review of articles. Iran J Neurol. 2014;13(4):209-214.
4. Dörr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T, et al. Characteristics of Susac syndrome: A review of all reported cases. Nat Rev Neurol. 2013;9(6):307-316. doi: 10.1038/nrneurol.2013.82
5. van der Kooij SM, van Buchem MA, Overbeek OM, Dijkman G, Huizinga TWJ. Susac syndrome: A report of four cases and a review of the literature. Neth J Med. 2015;73:10-16.