Neurosarcoidosis Masquerading as Giant Cell Arteritis With Incidental Meningioma

We present a case of vision loss secondary to neurosarcoidosis, which initially presented with severe bilateral vision loss, temporal headaches, and elevated erythrocyte sedimentation rate, concerning for giant cell arteritis. However, temporal artery biopsy was negative. Initial neuroimaging features were misinterpreted to represent a meningioma that did not account for his clinical presentation. Clinical course, including atypically rapid enlargement of presumed meningioma, development of skin lesions, appearance of optic nerve enhancement on MRI, and steroid response, strongly increased suspicion for sarcoidosis. Biopsy of a skin lesion demonstrated noncaseating granulomatous inflammation, consistent with sarcoidosis.

Photo Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Neurosarcoidosis Masquerading as Giant Cell Arteritis With Incidental Meningioma Omar M. Solyman, MD, Maria Adelita Vizcaino, MD, Roxana Fu, MD, Amanda D. Henderson, MD Downloaded from http://journals.lww.com/jneuro-ophthalmology by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= on 05/04/2022 FIG. 1. Contrast-enhanced T1 coronal (A) and axial (B) MRI of the orbits shows enhancing mass lesion arising from the region of the right cavernous sinus (arrows), suspicious for meningioma. Abstract: We present a case of vision loss secondary to neurosarcoidosis, which initially presented with severe bilateral vision loss, temporal headaches, and elevated erythrocyte sedimentation rate, concerning for giant cell arteritis. However, temporal artery biopsy was negative. Initial neuroimaging features were misinterpreted to represent a meningioma that did not account for his clinical presentation. Clinical course, including atypically rapid enlargement of presumed meningioma, development of skin lesions, appearance of optic nerve enhancement on MRI, and steroid response, strongly increased suspicion for sarcoidosis. Biopsy of a skin lesion demonstrated noncaseating granulomatous inflammation, consistent with sarcoidosis. Journal of Neuro-Ophthalmology 2021;41:e122–124 doi: 10.1097/WNO.0000000000000967 © 2020 by North American Neuro-Ophthalmology Society A 65-year-old African American man with a medical history of type 2 diabetes mellitus and hypertension presented with bilateral acute vision loss for 2 weeks, associated Wilmer Eye Institute (OMS, RF, ADH), Johns Hopkins University School of Medicine, Baltimore, Maryland; Department of Pathology (MAV), Division of Neuro-Ophthalmology (ADH, OMS); Division of Oculoplastics (RF), Johns Hopkins University School of Medicine, Baltimore, Maryland; and Department of Pathology, Mayo Clinic, Rochester, Minnesota. The authors report no conflicts of interest. Address correspondence to Amanda D. Henderson, MD, Wilmer Eye Institute, Johns Hopkins University School of Medicine, 600 N. Wolfe Street, Wilmer 233, Baltimore, MD 21287; E-mail: [e-mail] e122 with intermittent bitemporal headaches. At presentation, his visual acuity was counting fingers in the right eye and hand motion in the left with no relative afferent pupillary defect. His ocular examination was otherwise normal with no cause for the vision loss identified. Optical coherence tomography revealed normal, symmetric peripapillary retinal nerve fiber layer thicknesses. MRI of the brain and orbits showed a small dural-based focus of enhancement arising from the wall of the right cavernous sinus, suggestive of meningioma, but was negative for optic nerve enhancement or compression (Fig. 1). Due to initial concern for giant cell arteritis (GCA), the patient was admitted urgently for evaluation and treatment with intravenous methylprednisolone. He underwent extensive serum laboratory testing for potential infectious and inflammatory causes of bilateral vision loss, including GCA, systemic lupus erythematosus, sarcoidosis, neuromyelitis optica, granulomatosis with polyangiitis, IgG4-related disease, syphilis, tuberculosis, and Lyme disease. Abnormal serum results showed erythrocyte sedimentation rate of 77, mild anemia with hemoglobin 12.7, and positive ANA with titer 1:160. Unremarkable results included comprehensive metabolic panel, C-reactive protein, angiotensin-converting enzyme, lysozyme, serum and urine protein electrophoresis, immunoglobulins, aquaporin-4 antibody, anti-neutrophil cytoplasmic antibody, anti-SSA and SSB, anti-double stranded DNA, syphilis, t-spot, and Lyme antibodies. Chest x-ray showed no abnormalities. Cerebrospinal fluid studies were remarkable for slightly elevated protein of 56.9 mg/dL Solyman et al: J Neuro-Ophthalmol 2021; 41: e122-e124 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay FIG. 2. A. Contrast-enhanced T1 coronal MRI of the orbits shows subtle bilateral optic nerve enhancement (arrowheads). B. Axial cut demonstrates interval growth of the right paracavernous sinus mass lesion (arrow). (upper limit of normal 45). Temporal artery biopsy was negative. The patient was treated with 3 days of intravenous methylprednisolone, and then discharged on prednisone, pending the results of his temporal artery biopsy. While on steroid treatment, he experienced dramatic improvement of his vision to 20/20 in both eyes with full color vision and fields. Unfortunately, his steroid course was complicated by thoracic shingles and severely elevated blood glucose levels despite insulin use. He initially was placed on a slow steroid taper, but, due to his steroid complications and unclear diagnosis, the taper was accelerated. His visual function remained excellent for about 2 months, at which point he re-presented with right eye vision loss. He had no eye pain or headache at this time. His acuity was 20/50 in the right eye, with a right relative afferent pupillary defect and an inferior altitudinal field defect. Repeat MRI showed enlargement of the right paracavernous sinus mass lesion and subtle bilateral optic nerve enhancement (Fig. 2). Due to the rapid enlargement and indistinct borders of the presumed meningioma, as well as his response to steroid and recurrence on steroid withdrawal, sarcoidosis was suspected. Chest computed tomography showed bilateral hilar lymph node enlargement and peribronchovascular pulmonary nodules, supportive of that diagnosis. In the interval, his acuity unfortunately declined in both eyes, and he developed a cutaneous forehead lesion without evidence of dermatomal distribution or fungal cause (Fig. 3). Biopsy of the forehead lesion showed noncaseating granulomatous inflammation with multinucleated giant cells, consistent with sarcoidosis (Fig. 4). He resumed oral prednisone, with some visual improvement in the left eye but with residual counting fingers vision in the right eye. He has been maintained on prednisone and mycophenolate mofetil, with one relapse treated with IV steroid, without further visual loss. findings often mimic the appearance of other central nervous system diseases, including meningioma, infectious or carcinomatous meningitis, demyelinating disease, tuberculosis, granulomatosis with polyangiitis, lymphoma, or other neoplastic disease (1). We describe a case that initially presented with signs and symptoms suggestive of GCA, and in which a dural-based intracranial mass lesion initially was misdiagnosed as meningioma. The combination of clinical and neuroimaging findings, as well as the atypical behavior of the presumed meningioma, eventually led to the correct diagnosis of neurosarcoidosis. Neurosarcoidosis involving the visual pathways requires treatment with steroid, and sometimes additional immunosuppressive agents, to minimize visual morbidity (2). Therefore, a diagnosis of sarcoidosis should be considered in cases of presumed meningioma that appear atypical, either due to accelerated growth; unusual appearance, like the indistinct margins in this case; or associated findings, like the development of skin lesions and optic nerve enhancement in this case. Ideally, histopathologic diagnosis should be attained for confirmation of sarcoidosis because often these patients require long-term immunosuppression; however, in suspicious cases without an extracranial site of involvement, a trial of steroid may considered as a surrogate “diagnostic” procedure. INTERPRETATION OF FINDINGS The presentation of neurosarcoidosis can be highly variable and can mimic other inflammatory conditions, such as GCA. MRI Solyman et al: J Neuro-Ophthalmol 2021; 41: e122-e124 FIG. 3. External photograph shows erythematous forehead lesion with ulceration and crusting, extending on both sides of midline. e123 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay osis patients, may provide a potential low-morbidity biopsy site. The appearances of cutaneous sarcoidosis are myriad, but biopsy may demonstrate the noncaseating granulomas typical of sarcoidosis (3). Integration of multiple systemic findings including initially unexplained vision loss that improved with steroid treatment, an atypical intracranial meningioma, and the development of skin lesions, led to the diagnosis of multisystem sarcoidosis as the cause for vision loss in this case. FIG. 4. Biopsy of the forehead lesion shows noncaseating granulomatous inflammation with multinucleated giant cells, consistent with sarcoidosis. H&E ·100. Because the locations of neuro-ophthalmic sarcoidosis involvement often are not amenable to low-risk biopsy, neuro-ophthalmologists frequently search for pulmonary or other system involvement to facilitate histopathologic diagnosis. In this case, a minimally invasive elliptical biopsy of a skin lesion, performed under local anesthesia in the eye clinic, led to the histopathologic diagnosis. Although the pulmonary system is most commonly affected in sarcoidosis, the skin is the second most frequently involved body system (3). Evaluation for cutaneous involvement, which occurs in 20%–35% of sarcoid- e124 STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: O. M. Solyman and A.D. Henderson; b. Acquisition of data: O. M. Solyman, M.A. Vizcaino, R. Fu, and A.D. Henderson; c. Analysis and interpretation of data: O. M. Solyman, M.A. Vizcaino, and A.D. Henderson. Category 2: a. Drafting the manuscript: O. M. Solyman and A.D. Henderson; b. Revising it for intellectual content: O. M. Solyman, M.A. Vizcaino, R. Fu, and A.D. Henderson. Category 3: a. Final approval of the completed manuscript: O. M. Solyman, M.A. Vizcaino, R. Fu, and A.D. Henderson. REFERENCES 1. Ginat DT, Dhillon G, Almast J. Magnetic resonance imaging of neurosarcoidosis. J Clin Imaging Sci. 2011;1:15. 2. Koczman JJ, Rouleau J, Gaunt M, Kardon RH, Wall M, Lee AG. Neuro-ophthalmic sarcoidosis: the University of Iowa experience. Semin Ophthalmol. 2008;23:157–168. 3. Karadag AS, Parish LC. Sarcoidosis: a great imitator. Clin Dermatol. 2019;37:240–254. Solyman et al: J Neuro-Ophthalmol 2021; 41: e122-e124 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.