Helmchen C, Arbusow V, Jäger L, Strupp M, Stöcker W, Schulz P
Department of Neurology, University of Munich, Klinikum Grosshadern, Germany.
Acta Otolaryngol. 1999;119(5):528-36. doi: 10.1080/00016489950180748.
The aim of this study was to evaluate the pathological significance of antibodies against cornea and inner ear tissue in the development of audiovestibular and ocular symptoms in patients with Cogan's syndrome (CS). We analysed the serum of 5 CS patients for binding of IgM and IgG to fresh cryosections of rat labyrinth (semicircular canals, ampulla, utricle, saccule) and cornea by indirect immunofluorescence (IF). The predominant pattern of anti-corneal IgM was staining of the superficial cell layer of the non-keratinizing squamous epithelium. IgM against cornea was found in 3 patients, all of whom had bilateral inflammatory eye signs at the start of the disease. However, IgM was also detected in the chronic stage of the disease when no clinical signs of eye involvement were apparent. The study includes the first follow-up examination of anti-corneal IgM and IgG antibodies during a complete episode of active CS. During the first episode of CS in 1 patient, anti-corneal IgM became detectable 1 week after the onset of interstitial keratitis and 3 weeks after the onset of audiovestibular symptoms. It increased over several weeks and then fell to very low levels. However, at no time was anti-corneal IgG found. In the course of follow-up examinations, the serum of 4 patients intermittently contained low titre IgG antibodies against inner ear labyrinthine tissue, but without any clear correlation with the active stages of CS. In addition, high-resolution MRI (HR-MRI) of the inner ear was performed in the acute and chronic stages of CS to evaluate the activity of CS. In the acute stage, HR-MRI revealed abnormal MRI signals in the vestibule, semicircular canals, vestibular nerve, or cochlea. In the chronic stage, patients showed narrowing or occlusion of semicircular canals and the cochlea on the 3D-CISS images, but no high signal lesions (T1) and no enhancement. Antibodies against cornea or labyrinthine tissue were not consistently detected in CS and the level of organ-specific antibodies did not correlate with the activity of the disease.
本研究的目的是评估抗角膜和内耳组织抗体在柯根综合征（CS）患者听觉前庭和眼部症状发展中的病理意义。我们通过间接免疫荧光法（IF）分析了5例CS患者血清中IgM和IgG与大鼠迷路（半规管、壶腹、椭圆囊、球囊）和角膜新鲜冰冻切片的结合情况。抗角膜IgM的主要模式是非角化鳞状上皮浅层细胞层染色。3例患者检测到抗角膜IgM，所有患者在疾病开始时均有双侧眼部炎症体征。然而，在疾病的慢性期也检测到了IgM，此时眼部受累的临床体征并不明显。该研究包括在活动性CS的整个病程中首次对抗角膜IgM和IgG抗体进行随访检查。在1例患者的CS首次发作期间，抗角膜IgM在间质性角膜炎发作1周后和听觉前庭症状发作3周后可检测到。它在数周内升高，然后降至非常低的水平。然而，未发现抗角膜IgG。在随访检查过程中，4例患者的血清间歇性地含有低滴度抗内耳迷路组织IgG抗体，但与CS的活动阶段无明显相关性。此外，在CS的急性期和慢性期对内耳进行了高分辨率MRI（HR-MRI）检查，以评估CS的活动情况。在急性期，HR-MRI显示前庭、半规管、前庭神经或耳蜗的MRI信号异常。在慢性期，患者在3D-CISS图像上显示半规管和耳蜗变窄或闭塞，但无高信号病变（T1）且无强化。CS患者中并非始终能检测到抗角膜或迷路组织抗体，器官特异性抗体水平与疾病活动度无关。
