Susac's syndrome during pregnancy — The first Croatian case

• Published in: Journal of the neurological sciences 2014-06, Vol.341 (1), p.162-164
• Main Authors: Antulov, Ronald, Holjar Erlic, Izidora, Perkovic, Olivio, Miletic, Damir, Antoncic, Igor
• Format: Article
• Language: English
• Subjects: Corpus callosum; Diffusion Magnetic Resonance Imaging; Encephalopathy; Female; Hearing loss; Humans; Immunosuppressive therapy; Internal Capsule - pathology; Magnetic Resonance Imaging; Neurology; Pregnancy; Susac Syndrome - diagnosis; Susac's syndrome; Young Adult
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2014.04.010

Abstract Susac's syndrome (SS) is an infrequent neurological disorder characterized by the clinical triad of encephalopathy, branch retinal artery occlusion and hearing loss due to an autoimmune endotheliopathy associated with anti-endothelial cell antibodies. At the onset of the disease SS rarely appears with the complete clinical triad. The most important diagnostic procedures involved in the diagnosis of SS are brain MRI, audiometric testing and retinal fluorescein angiography. Presence of at least two components of the SS clinical triad accompanied by specific brain MRI findings is highly suggestive of SS. We report a case of a young pregnant woman with a history of encephalopathy, hearing loss and walking impairment. Brain MRI revealed a spectrum of findings previously described in patients with SS. We induced labor at 37 weeks' gestation to start with immunosuppressive treatment and avoid possible fetal toxicity. To the best of our knowledge this is the first report of SS in Croatia.