Nazliel Bijen, Akyol Asli, Zeynep Batur Caglayan Hale, Yildirim-Capraz Irem, Irkec Ceyla
Department of Neurology, Faculty of Medicine, Gazi University, 06500 Ankara, Turkey.
Case Rep Neurol Med. 2014;2014:214648. doi: 10.1155/2014/214648. Epub 2014 Feb 2.
Susac's syndrome is an uncommon neurologic disorder of unknown cause. It has been described as a clinical triad of encephalopathy, hearing loss, and branch retinal artery occlusions. Clinically the diagnosis is difficult when the patient presents only a portion of a triad. We present a case with vision loss and sensorineural deafness and who had been diagnosed with MS for 20 years. Susac's syndrome is presumed to be an autoimmune endotheliopathy. Neurologic symptoms and signs are diffuse and multifocal, acute or subacute in onset, and progress during the active phase of the disease. In some patients the onset was stroke like and in others that of subacute dementia. Headache, often with migrainous features, was a prominent feature initially in more than one half of the patients. A high index of suspicion leading to correct diagnosis and early appropriate therapy may reduce the permanent sequel seen with this disease. Misdiagnosis is common. In patients in whom diagnosis and treatment are delayed permanent morbidity is higher in terms of visual loss, hearing loss, and neurologic debility. In patients in whom rapid diagnosis has led to early administration of immunosuppressive therapy, recovery can be almost complete.
苏萨克综合征是一种病因不明的罕见神经系统疾病。它被描述为脑病、听力丧失和视网膜分支动脉阻塞的临床三联征。临床上，当患者仅表现出三联征的一部分时，诊断较为困难。我们报告一例视力丧失和感音神经性耳聋的病例，该患者已被诊断为多发性硬化症20年。苏萨克综合征被推测为一种自身免疫性内皮病变。神经系统症状和体征弥漫且多灶性，起病急或亚急性，在疾病活动期进展。在一些患者中，起病类似中风，而在另一些患者中则类似亚急性痴呆。头痛通常具有偏头痛特征，最初在超过一半的患者中是突出特征。高度怀疑从而导致正确诊断和早期适当治疗，可能会减少该疾病所见的永久性后遗症。误诊很常见。在诊断和治疗延迟的患者中，就视力丧失、听力丧失和神经功能衰弱而言，永久性发病率更高。在快速诊断并早期给予免疫抑制治疗的患者中，恢复几乎可以完全。
