Sader A, Gramer E, Körner U
Universitätsaugenklinik Würzburg.
Klin Monbl Augenheilkd. 1995 Feb;206(2):128-33. doi: 10.1055/s-2008-1035416.
Rarely an affection of the optic nerve is seen as the initial or only manifestation of sarcoidosis. Therefore the disease is often diagnosed late. The aim of systemic corticosteroid therapy is to prevent progression though it cannot yield a cure.
Despite a high-dose corticosteroid therapy in suspected optic neuritis a 25-year-old woman developed unilateral amaurosis. When visual acuity continuously decreased in the second eye a computerized tomography was performed, which suggested a tumor of the optic nerve. A biopsy of this lesion lead to the diagnosis of Boeck's disease. A long-term corticosteroid therapy was initiated. Over the following 9-year period corticosteroids were dosed according to the results of regular clinical and perimetrical examinations (200 examinations with Octopus-Perimeter 201, program G1). In case of deterioration of the visual field higher oral doses were applied. When no improvement was achieved by this, corticosteroids were given intrathecally. Under this therapeutic regime no systemic side effects were seen.
In unilateral visual loss Boeck's disease should be considered as a rare etiology. In case of clear optical media frequent computerized perimetry allows the neurologist to adjust the dosage of cortisone and minimize its side effects.
视神经受累很少作为结节病的初始或唯一表现出现。因此，该病常被延迟诊断。全身糖皮质激素治疗的目的是防止病情进展，尽管无法治愈。
一名25岁女性尽管在疑似视神经炎时接受了高剂量糖皮质激素治疗，但仍出现单侧黑矇。当另一只眼视力持续下降时，进行了计算机断层扫描，提示视神经肿瘤。对该病变进行活检后诊断为伯克氏病。开始长期糖皮质激素治疗。在接下来的9年里，根据定期临床和视野检查结果（使用Octopus-Perimeter 201，程序G1进行了200次检查）调整糖皮质激素剂量。如果视野恶化，则增加口服剂量。若以此方法未取得改善，则鞘内注射糖皮质激素。在这种治疗方案下未观察到全身副作用。
在单侧视力丧失的情况下，应将伯克氏病视为一种罕见病因。在光学介质清晰的情况下，频繁进行计算机视野检查可使神经科医生调整可的松剂量并将其副作用降至最低。
