Jacobson D M
Department of Neurology, Marshfield Clinic, Wisconsin 54449, USA.
J Neuroophthalmol. 1996 Sep;16(3):172-7.
This case report describes a patient who developed the characteristic features of acute zonal occult outer retinopathy (AZOOR), but whose case was unusual because of macular involvement, recurrences, and association with an inflammatory CNS disorder. The patient was followed for > 7 years; clinical examinations, electroretinography, electro-oculography, cerebrospinal fluid evaluations, and magnetic resonance imaging (MRI) were used to document the recurrent AZOOR and CNS disorder. The patient first presented with entoptic symptoms and a scotoma referable to dysfunction of her peripapillary and peripheral right retina. At that time, she also had asymptomatic involvement of her peripheral left retina, cerebrospinal fluid pleocytosis, and multiple brain MRI signal abnormalities. During the next several years, she developed recurrences of AZOOR, which first affected her right macula and later involved her peripheral left retina. Visual electrophysiological studies confirmed impairment of outer retinal function. The patient developed her first neurological symptom, acute relapsing-remitting cervical myelitis, > 6 years after her visual presentation. Her clinical course, laboratory studies, and neurodiagnostic evaluations were consistent with CNS inflammation, but they were not typical of multiple sclerosis. Since AZOOR is a newly recognized disorder, its full clinical spectrum may not yet be established and could include CNS involvement.
本病例报告描述了一名出现急性区域性隐匿性外层视网膜病变（AZOOR）特征性表现的患者，但该病例因黄斑受累、复发以及与中枢神经系统炎性疾病相关而显得不同寻常。对该患者进行了7年多的随访；采用临床检查、视网膜电图、眼电图、脑脊液评估以及磁共振成像（MRI）来记录复发性AZOOR和中枢神经系统疾病。患者最初表现为视幻觉症状以及与右视乳头周围和周边视网膜功能障碍相关的暗点。当时，她的左周边视网膜也有无症状受累，脑脊液淋巴细胞增多，以及多个脑部MRI信号异常。在接下来的几年里，她出现了AZOOR复发，最初影响其右黄斑，随后累及左周边视网膜。视觉电生理研究证实外层视网膜功能受损。患者在出现视觉症状6年多后出现了首个神经系统症状，即急性复发 - 缓解型颈髓炎。她的临床病程、实验室检查以及神经诊断评估与中枢神经系统炎症相符，但并非典型的多发性硬化症。由于AZOOR是一种新认识的疾病，其完整的临床谱可能尚未确立，并且可能包括中枢神经系统受累。
