Boschetti Mara, De Lucchi Massimo, Giusti Massimo, Spena Claudio, Corallo Guido, Goglia Umberto, Ceresola Enrica, Resmini Eugenia, Vera Lara, Minuto Francesco, Ferone Diego
Department of Endocrinological and Metabolic Sciences and Center of Excellence for Biomedical Research, University of Genoa ans San Martino Hospital, Italy.
Eur J Endocrinol. 2006 Jun;154(6):813-8. doi: 10.1530/eje.1.02161.
Here we describe the case of a 41-year-old woman with a history of Cushing disease who had previously undergone unsuccessful neurosurgery, followed by stereotactic radiosurgery. More than 4 years after this treatment, she presented severe visual impairment, which started in the left eye and was documented by neuro-ophthalmic evaluation. Radiological assessment by contrast-enhanced magnetic resonance (MR) imaging initially suggested the diagnosis of glioma of the optic nerve and the patient started corticosteroid treatment (first with prednisone, 80 mg/day, followed by dexamethasone, 8 mg/day). Despite the therapy, vision in the left eye rapidly worsened until light was no longer perceptible; similar symptoms and signs also developed in the right eye, evolving to complete temporal hemianopsia. The clinical evidence was confirmed by the rapid progression of the MR picture, which showed homogeneous enhancement of the chiasm and optic nerves. On the basis of these findings, the original diagnosis of glioma was excluded, and radiation-induced optic neuropathy was diagnosed. As corticosteroids had proved inefficacious, hyperbaric oxygen (HBO) therapy was promptly instituted and vision steadily started to improve. This improvement was documented and confirmed by the progressive recovery of the visual field in the right eye and the changes in the sequential follow-up MR scanning. Optic neuropathy is an infrequent but dramatic complication of radiation therapy. Symptoms develop, on average, 12 months after treatment, and the onset may be acute and characterized by the progressive loss of vision in one or both eyes. HBO has already been used to treat this complication, but its efficacy is still controversial. Here, in addition to describing this particular case, which presented a significantly delayed radiation injury of the optic pathways, we provide a brief literature review and discuss some important points.
在此,我们描述了一名41岁患有库欣病的女性病例,她此前接受过神经外科手术但未成功,随后接受了立体定向放射外科治疗。在该治疗4年多后,她出现了严重的视力损害,始于左眼,并经神经眼科评估记录。对比增强磁共振(MR)成像的放射学评估最初提示诊断为视神经胶质瘤,患者开始接受皮质类固醇治疗(首先使用泼尼松,80毫克/天,随后使用地塞米松,8毫克/天)。尽管进行了治疗,左眼视力仍迅速恶化直至无光感;右眼也出现了类似症状和体征,发展为完全性颞侧偏盲。MR图像的快速进展证实了临床证据,该图像显示视交叉和视神经均匀强化。基于这些发现,排除了最初的胶质瘤诊断,诊断为放射性视神经病变。由于皮质类固醇已被证明无效,遂立即开始高压氧(HBO)治疗,视力开始稳步改善。右眼视野的逐渐恢复以及后续连续MR扫描的变化记录并证实了这种改善。视神经病变是放射治疗罕见但严重的并发症。症状平均在治疗后12个月出现,发病可能很突然,表现为一只或两只眼睛视力逐渐丧失。HBO已被用于治疗这种并发症,但其疗效仍存在争议。在此,除了描述这个出现明显延迟的视路放射性损伤的特殊病例外,我们还提供了简要的文献综述并讨论了一些要点。