Contemporary view of the pathogenesis, diagnosis and treatment of Susac syndrome
Chukhlovina M.L.¹, Voznyuk I.A.², Polushin A.Yu.<sup>3,4

1</sup> Almazov National Medical Research Centre, Saint-Petersburg
² Medical Military Academy named after S.M. Kirov, Saint-Petersburg
³ Pavlov First Saint Petersburg State Medical University
⁴ Raisa Gorbacheva Memorial Research Institute for Pediatric Oncology, Hematology and Transplantation, Saint-Petersburg

Susaс syndrome is an immune-mediated disease of the nervous system. In the clinical picture, it is represented by a triad of the main symptoms: subacute encephalopathy, occlusion of the retinal artery branches and neurosensory hearing loss. One of the main symptoms, in addition to visual impairment, may be a severe headache. These symptoms are based on microangiopathy. The immune-associated endotheliopathy in the microvascular channel leads to hypertrophy of endothelial cells with the subsequent occlusion of arterioles. As a result, there is perivascular inflammation followed by ischemia of the brain and retina. Differential diagnosis of the syndrome should be carried out with demyelinating diseases, vasculitis, toxic leukoencephalopathy. Blood and cerebrospinal fluid tests in most cases are not informative. The main role in diagnosing Susaс syndrome belongs to magnetic resonance imaging, according to which changes in the type of «snow balls» mainly in the central part of the corpus callosum are detected. The use of pulse therapy with methylprednisolone usually does not lead to the proper effect. This involves, along with the introduction of corticosteroids, the use of cytostatics and intravenous immunoglobulins. The scheme of therapy escalation of Susaс syndrome is presented in this article.

Key words: Susac syndrome, RED-M, SICRET, autoimmune diseases, microangiopathy, vasculitis, subacute encephalopathy, occlusion of the retinal artery branches, neurosensory hearing loss.
For citation: Chukhlovina M.L., Voznyuk I.A., Polushin A.Yu. Contemporary view of the pathogenesis, diagnosis and treatment of Susac syndrome // RMJ. 2018. № 7. P. 10–14.