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Non-DR Retinal Vascular Diseases
Published in Ching-Yu Cheng, Tien Yin Wong, Ophthalmic Epidemiology, 2022
Sobha Sivaprasad, Luke Nicholson, Shruti Chandra
Anecdotal reports of central branch or cilioretinal artery occlusion associated with Fabry’s disease, incontinentia pigmenti, cocaine usage, migraine, Marfan’s syndrome, nephrotic syndrome, and snake bite have been made (114–119). There are numerous case reports of Susac syndrome (retinocochleocerebral syndrome), which is a triad of encephalopathy, cochlear hearing loss, and BRAO (120).
Paper 3
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Susac syndrome has a preference for the corpus callosum with lesions affecting the body and splenium. There tend to be multiple small lesions which resemble ‘snowballs’ on T2 sequences. The other radiological features of excess alcohol intake in this case make Marchiafava-Bignami more likely. The clinical history in Susac syndrome is usually one consistent with brain, retinal and vestibulocochlear involvement.
What is the potential of paramagnetic rim lesions as diagnostic indicators in multiple sclerosis?
Published in Expert Review of Neurotherapeutics, 2022
Maria Sofia Martire, Lucia Moiola, Maria Assunta Rocca, Massimo Filippi, Martina Absinta
The only exception is represented by Susac’s syndrome that, unlike other inflammatory neurological diseases, features non-perivenular lesions in the corpus callosum resembling PRL. Susac’s syndrome is a rare vasculitis of small vessels, which affects the retina, the inner ear, the cerebral parenchyma, and the meninges. In the three available studies on this topic [33,37,57], 11 Susac’s patients were studied and 7 of them had PRL (63.6%). However, only 4% of total white matter lesions showed a paramagnetic rim [57]. It has been proposed that the susceptibility MRI findings in Susac’s syndrome may be due to the microangiopathy-related blood extravasation and accumulation of iron from hemoglobin degradation [37]. However, Susac’s syndrome holds unique clinical features (such as neurosensorial hearing loss and branch retinal artery occlusion) [66] as well as distinct radiological patterns [66], which are not typical of MS.
Brain microvascular pathology in Susac syndrome: an electron microscopic study of five cases
Published in Ultrastructural Pathology, 2019
Dimitri P. Agamanolis, Richard A. Prayson, Negar Asdaghi, Sakir H. Gultekin, Kim Bigley, Robert M. Rennebohm
Susac Syndrome (SuS) is a rare, immune-mediated disease that affects the microvasculature of the brain, retina, and inner ear. It predominantly affects young women (age 20–40 years, mean age of 32) and is characterized by a clinical triad of encephalopathy, branch retinal artery occlusion (BRAO), and sensorineural hearing loss.1-4 Classically, MRI shows “snowball” lesions in the central portion of the corpus callosum. Extra-callosal lesions are also seen. Affecting patients at such young age, SuS has the potential to cause dementia, blindness, profound deafness, severe life-long disability, and death. Criteria have been proposed for diagnosis of definite, probable, and possible SuS.4 Since the majority of patients with SuS do not exhibit all three components of the clinical triad at the time of presentation, and since some patients have atypical features, diagnosis is often delayed and/or in doubt. In such instances, it is appropriate to consider brain biopsy to help clarify diagnosis.
28-year-old male anabolic steroid abuser with Susac syndrome. An interdisciplinary case report
Published in Modern Rheumatology Case Reports, 2019
Natalia Lewczuk, Alexander Zdebik, Joanna Bogusławska, Magdalena Targońska, Anna Turno-Kręcicka
Susac syndrome is believed to be an immunological endotheliopathy that affects the microvasculature of the brain, retina and inner ear. However, other theories such as vasospasm, coagulation disorders, and viral infections have also been considered [8]. In our case, the patient had an unknown viral infection, which could be a trigger of the syndrome. We cannot exclude the influence of anabolic steroids on the development of Susac syndrome in our case. The pathophysiological effects of using anabolic steroids are well-known and include an increase in renal erythropoietin synthesis stimulating haematopoiesis, lipolysis, protein synthesis, sebaceous secretion and hair growth [9]. Erythropoietin, according to a latest study, has effects not only on blood production but is also an angiogenetic factor and has influence on endothelial progenitor cells [10]. Erythropoetin receptors are found amongst others in tissues like the retina, inner ear and central nervous system, where an endotheliopathy in the triad of Susac syndrome is observed [11–13]. Erythropoetin is being discussed as a factor positively influencing anti-inflammatory, anti-apoptotic and neuroprotective processes but also as a factor negatively influencing the development of retinopathy of prematurity [14]. Only further observations will show if anabolic steroids have an influence on the development of Susac syndrome. This is the first case of Susac syndrome that is potentially caused by anabolic steroids and its subsequent effect on erythropoietin synthesis, thus inducing Susac syndrome. A similar phenomenon describing the induction of an inflammatory reaction mediated by testosterone, more specifically anaemia of inflammation through an autoimmune process has been described in a female mouse model [15].