Identifier |
walsh_2024_s2_c3 |
Title |
Double Double, Toil and Trouble |
Creator |
Kevin Yan; Adeniyi Fisayo; Joachim Bähring; Declan McGuone |
Affiliation |
(KY) (AF) (JB) (DM) Yale University |
Subject |
Diplopia; Cavernous Sinus Syndrome |
Description |
Our patient initially presented in 2013 (age 66 years) with progressive subacute blurry vision, that progressed to double vision. She has history notable for remote breast cancer status post chemoradiation, in remission since 2001. She reported binocular double vision, worsened when looking left or down, included both horizontal and diagonal components, and generally worse with distance. She did not have headache or other neurologic symptoms. Neurologic examination showed mild left exophthalmos and deficits consistent with left trochlear and abducens nerve palsies. Brain MRI with and without contrast (Image 1) showed an enhancing left cavernous sinus mass, which was thought to be a meningioma. Biopsy was deferred due to concern for neurologic morbidity, and she underwent empiric radiation with improvement. In 2018 she reported jaw numbness. Brain MRI showed progression of the previously described lesion in the left cavernous sinus (Image 2). She was again treated with radiation and her symptoms improved. In late 2022, she reported horizontal double vision and lower motor neuron pattern left facial weakness. Imaging showed significant disease progression compared to 2018 with multiple new lesions and interval development of pachymeningeal enhancement (old lesion Image 3; new lesions 4, 5, 6); spinal imaging showed epidural enhancement and a L5 osseus lesion (Image 7). Extensive evaluation for malignancy (CT chest/abdomen/pelvis, PET scan, bone scan, serologic studies including flow cytometry) was completed without evidence of systemic illness. The patient had two biopsies in 2022 and early 2023 of the two peripheral lesions, which were read as having chronic inflammatory changes without clear disease. Throughout this time, the patient has continued to experience symptoms and morbidity related primarily to her double vision. |
History |
The patient's symptoms are all related to her multiple intracranial lesions, with the left cavernous sinus lesion being the most symptomatic and resulting double vision and jaw numbness. The lower motor neuron pattern left facial weakness is likely attributable to posterior extension of the lesion along the skull base to involve the facial nerve in the internal auditory canal. The crux of this case was figuring out why she had multiple slow-growing intracranial masses. It was also challenging to differentiate disease progression from possible adverse effects of radiotherapy. A final biopsy performed by otolaryngology of the left inferior fossa tumor was performed (see Pathology images). This was notable for histiocytosis with emperipolesis. Notably, a large majority of these histiocytes stained positive for S100 protein. Stains for other diseases, including IgG4 disease, were negative. The patient was diagnosed with an underlying histiocytic disorder, Rosai-Dorfman syndrome. Once the diagnosis was made, the patient consulted with a hematologist/oncologist and was prescribed a prolonged course of systemic corticosteroid. Rosai-Dorfman syndrome (originally reported as sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman-Destombes disease), first described in 1969, is a histiocytosis that occurs due to excessive histiocyte production in the lymph nodes, causing accumulation in other parts of the body, most commonly the cervical lymph nodes, but 40% of patients will have disease involvement outside the lymph nodes. This is typically a slow progressing disorder that is typically benign, and most cases undergo spontaneous remission. Treatment is reserved for symptomatic cases only and is usually limited to corticosteroids. |
Disease/Diagnosis |
Progressive unilateral cranial nerve palsies from a skull base lesion that was ultimately identified as Rosai- Dorfman syndrome. |
Date |
2024-03 |
References |
Foucar E, Rosai J, Dorfman RD: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Review of the entity. Semin Diagn Pathol 7:19-73, 1990 Foucar E, Rosai J, Dorfman RF, et al: The neurologic manifestations of sinus histiocytosis with massive lymphadenopathy. Neurology 32:265-371, 1982 Foucar E, Rosai J, Dorfman RF: The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. Am J Opthalmo 187:354-367, 1979 Oussama Abla, Eric Jacobsen, Jennifer Picarsic, Zdenka Krenova, Ronald Jaffe, Jean-Francois Emile, Benjamin H. Durham, Jorge Braier, Frédéric Charlotte, Jean Donadieu, Fleur Cohen-Aubart, Carlos Rodriguez-Galindo, Carl Allen, James A. Whitlock, Sheila Weitzman, Kenneth L. McClain, Julien Haroche, Eli L. Diamond; Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease. Blood 2018; 131 (26): 2877-2890. doi: https://doi.org/10.1182/blood- 2018-03-839753 McClain KL, Bigenwald C, Collin M, Haroche J, Marsh RA, Merad M, Picarsic J, Ribeiro KB, Allen CE. Histiocytic disorders. Nat Rev Dis Primers. 2021 Oct 7;7(1):73. doi: 10.1038/s41572-021-00307-9. PMID: 34620874; PMCID: PMC10031765. |
Language |
eng |
Format |
video/mp4 |
Type |
Image/MovingImage |
Source |
2024 North American Neuro-Ophthalmology Society Annual Meeting |
Relation is Part of |
2024 Frank B. Walsh Sessions: Session II |
Collection |
Neuro-Ophthalmology Virtual Education Library: NANOS Annual Meeting Collection: https://novel.utah.edu/collection/nanos-annual-meeting-collection/ |
Publisher |
North American Neuro-Ophthalmology Society |
Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
Rights Management |
Copyright 2024. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
ARK |
ark:/87278/s6wxk6qq |
Setname |
ehsl_novel_fbw |
ID |
2592821 |
Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6wxk6qq |