Division of Neuro-Ophthalmology (VPD, KAAD, BKC), Massachusetts Eye and Ear/Harvard Medical School, Boston, Massachusetts; and Departments of Radiology (OR), Pathology (SNC), and Neurology (BKC), Massachusetts General Hospital/Harvard Medical School, Boston, Massachusetts.
J Neuroophthalmol. 2021 Mar 1;41(1):114-118. doi: 10.1097/WNO.0000000000001141.
A 44-year-old woman presented with 2 painful and self-limited episodes of binocular horizontal diplopia within 1 year that at the beginning were thought to be secondary to microvascular insult. Her medical history was significant for Cushing syndrome status post transsphenoidal resection with bilateral adrenalectomy 4 years prior, hypertension, and diabetes mellitus. Neuro-ophthalmic evaluation was significant for left abduction deficit and incomitant esotropia consistent with left abducens nerve palsy. Of note, the patient had experienced a similar episode but on the contralateral side a few months prior. Although initially MRI of the brain demonstrated stable residual postoperative finding in the sella, upon review, an heterogenous T-1 hypointense marrow in the clivus was noted. Hypermetabolism of the clivus was also noted on computed tomography positron emission tomography of the skull base. A clival biopsy demonstrated a corticotroph adenoma with elevated proliferation index and scattered mitoses. A corticotroph pituitary adenoma after adrenalectomy, also known as Nelson syndrome, was diagnosed. Radiation therapy was offered to the patient, and resolution of symptoms was gradually observed.
一位 44 岁女性，在 1 年内出现 2 次双侧水平复视的疼痛性和自限性发作，最初认为是微血管损伤引起的。她的病史包括 4 年前经蝶窦切除术和双侧肾上腺切除术治疗库欣综合征、高血压和糖尿病。神经眼科评估显示左侧外展功能障碍和斜视不一致，符合左侧展神经麻痹。值得注意的是，患者曾有过类似的发作，但在几个月前对侧出现。尽管最初的脑部 MRI 显示鞍内术后残留的稳定发现，但在复查时，注意到斜坡的 T1 低信号不均匀骨髓。颅底计算机断层扫描正电子发射断层扫描也显示斜坡代谢亢进。斜坡活检显示为促肾上腺皮质激素腺瘤，增殖指数升高，有散在的有丝分裂。诊断为肾上腺切除术后的促肾上腺皮质激素垂体腺瘤，也称为纳尔逊综合征。向患者提供了放射治疗，症状逐渐缓解。
