TY - JOUR AU - Engeholm, Maik AU - Leo-Kottler, Beate AU - Rempp, Hansjörg AU - Lindig, Tobias AU - Lerche, Holger AU - Kleffner, Ilka AU - Henes, Melanie AU - Dihné, Marcel PY - 2013 DA - 2013/11/25 TI - Encephalopathic Susac’s Syndrome associated with livedo racemosa in a young woman before the completion of family planning JO - BMC Neurology SP - 185 VL - 13 IS - 1 AB - Susac’s Syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusions (BRAO) and hearing loss (HL). Histopathologically, SS is characterised by a microangiopathy, and some observations suggest that an immune-mediated damage of endothelial cells might play a role. These findings also implicate a similarity between SS and other autoimmune diseases, most notably juvenile dermatomyositis (JDM). However, SS and JDM are commonly thought to affect distinct and non-overlapping sets of organs, and it is currently not clear how these specificities arise. Moreover, in the absence of clinical trials, some authors suggest that therapeutic approaches in SS should rely on the model of other autoimmune diseases such as JDM. SN - 1471-2377 UR - https://doi.org/10.1186/1471-2377-13-185 DO - 10.1186/1471-2377-13-185 ID - Engeholm2013 ER -