Identifier |
walsh_2013_s4_c2 |
Title |
Occam's Razor or Gamma Knife? |
Creator |
Kaushal M. Kulkarni; Linda Sternau; Byron L. Lam |
Affiliation |
(KMK) (BLL) Department of Ophthalmology, University of Miami/Bascom Palmer Eye Institute, Miami, FL; (LS) Department of Neurosurgery, Memorial Neuroscience Center, Memorial Regional Hospital, Hollywood, FL |
Subject |
Wegener's Granulomatosis; Prolactinoma; Headaches; Magnetic resonance imaging; Intracranial tumors |
Description |
The patient was lost to follow-up for six months. She then presented again with pain and visual loss in the right eye. Visual acuity had decreased to 20/400 OD. Repeat MRI showed increased size of the mass and new involvement of the right pre-chiasmal optic nerve. A fronto-orbital craniotomy and decompression of the mass was performed. Pathology revealed fibrous tissue with an extensive inflammatory infiltrate, including granulomas, histiocytes, and scattered giant cells, along with perivascular inflammation and necrosis. The results were consistent with a diagnosis of Wegener's granulomatosis (WG). Upon further questioning, the patient revealed a history of a kidney biopsy showing WG over 12 years prior as the cause for her end-stage renal disease. She was placed on oral prednisone due to an adverse reaction to rituximab. Compliance and follow-up remain poor. Visual acuity at the last follow-up was 20/50 OD with an unchanged motility exam. WG is a systemic, necrotizing, granulomatous vasculitis of unknown etiology, first described in 1939 [1]. Diffuse small and medium size vessel involvement in WG classically leads to upper and lower respiratory tract, and kidney involvement. Central nervous system involvement has been reported in approximately 22-54% of cases of WG [2]. Most commonly this involves peripheral neuropathy resulting from vasculitis affecting the small vessels. Pituitary involvement is extremely rare, and the exact mechanism is unknown. When it does occur, it usually leads to central diabetes insipidus with loss of the normal hyperintense signal in the posterior pituitary. However, we are not aware of any similar reports of WG mimicking a pituitary prolactinoma [2,3]. |
History |
Hypertension, end-stage renal disease on hemodialysis, and an episode of anterior uveitis in the right eye 10 years earlier. |
Pathology |
Pathology revealed fibrous tissue with an extensive inflammatory infiltrate, including granulomas, histiocytes, and scattered giant cells, along with perivascular inflammation and necrosis. The results were consistent with a diagnosis of Wegener's granulomatosis (WG). |
Disease/Diagnosis |
Wegener's Granulomatosis Masquerading as a Prolactinoma |
Clinical |
Diagnosed with a right 3rd and 6th nerve palsy. |
Presenting Symptom |
A 53 year-old female presented to the ER with ptosis in right eye and double vision. |
Neuroimaging |
MRI |
Date |
2013-02 |
References |
1. Wegener F. About a peculiar rhinogenic granulomatosis with marked involvement of the arterial system and kidneys. Beitr Pathol Anat 1939;20;30-68. 2. Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE. Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol 1993;33:4-9. 3. HoffmanGS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116:488-98. |
Language |
eng |
Format |
video/mp4 |
Type |
Image/MovingImage |
Source |
45th Annual Frank Walsh Society Meeting |
Relation is Part of |
NANOS Annual Meeting 2013 |
Collection |
Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
Publisher |
North American Neuro-Ophthalmology Society |
Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
Rights Management |
Copyright 2013. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
ARK |
ark:/87278/s6s2104h |
Setname |
ehsl_novel_fbw |
ID |
179238 |
Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6s2104h |