Wilder-Smith E, Roelcke U
Department of Neurology, University of Bern, Switzerland.
J Clin Neuroophthalmol. 1990 Dec;10(4):261-5.
Cogan's syndrome is a rare systemic autoimmune disease with preeminent ophthalmological and vestibulocochlear manifestations. Untreated, the disease usually results in profound deafness; eye involvement is usually self-remitting. Ten to twenty percent of patients either develop serious aortic valve disease or vasculitis or both. When given early, immunosuppressive therapy has been shown to be effective in treating all aspects of the disease. Thus, early disease recognition is of great significance. We present a patient with Cogan's syndrome and briefly discuss therapeutic implications.
科根综合征是一种罕见的全身性自身免疫性疾病,以突出的眼科和前庭蜗症状为表现。若不治疗,该病通常会导致严重耳聋;眼部受累通常可自行缓解。10%至20%的患者会出现严重的主动脉瓣疾病或血管炎,或两者皆有。早期给予免疫抑制治疗已被证明对治疗该病的各个方面均有效。因此,早期识别疾病具有重要意义。我们报告一例科根综合征患者,并简要讨论其治疗意义。