Nelson Syndrome: Clival Invasion of Corticotroph Pituitary Adenoma Resulting in Alternating Sixth Nerve Palsies

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Title Nelson Syndrome: Clival Invasion of Corticotroph Pituitary Adenoma Resulting in Alternating Sixth Nerve Palsies
Creator Vivian P. Douglas; Konstantinos A. A. Douglas; Otto Rapalino; Samantha N. Champion; Bart K. Chwalisz
Affiliation 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
Abstract 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.
Subject ACTH-Secreting Pituitary Adenoma / pathology; ACTH-Secreting Pituitary Adenoma / surgery; Abducens Nerve Diseases / diagnosis; Adenoma / pathology; Adenoma / surgery; Adrenalectomy; Adrenocorticotropic Hormone / blood; Adult; Cranial Fossa, Posterior / pathology; Diplopia / diagnosis; Female; Humans; Magnetic Resonance Imaging; Nelson Syndrome / diagnosis; Neoplasm Invasiveness; Skull Base Neoplasms / pathology; Tomography, X-Ray Computed
OCR Text Show
Date 2021-03
Language eng
Format application/pdf
Type Text
Publication Type Journal Article
Source Journal of Neuro-Ophthalmology, March 2021, Volume 41, Issue 1
Collection Neuro-Ophthalmology Virtual Education Library: Journal of Neuro-Ophthalmology Archives: https://novel.utah.edu/jno/
Publisher Lippincott, Williams & Wilkins
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management © North American Neuro-Ophthalmology Society
ARK ark:/87278/s62mvpqr
Setname ehsl_novel_jno
ID 1765141
Reference URL https://collections.lib.utah.edu/ark:/87278/s62mvpqr
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