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A 55-year-old white woman presented with 1 week of decreased visual acuity and ache on abduction in the right eye. She had decreased right-sided hearing and tinnitus, a moderate right-sided frontal headache, and an unsteady gait. She did not have diplopia, facial numbness, or paresthesias. She had a history of stage IV breast cancer treated with letrozole after a mastectomy 5 years prior. On an initial examination, her best-corrected visual acuity was 20/50 OD and 20/25 OS, with dyschromatopsia, relative afferent pupillary defect, and visual-field defects on the right side. Her extraocular movements were full. A dilated fundus examination demonstrated a pale right optic nerve. Magnetic resonance imaging (MRI) of the brain was reported to have normal results, but on review, we noted thickening and enhancement of both optic nerves (with the right eye larger than the left eye) (Figure 1A), cranial nerve III in the right eye, cranial nerve V bilaterally, the pituitary infundibulum, and the hypothalamus, as well as adjacent cavernous sinuses. Leptomeningeal thickening over the convexities of the anterior frontal lobes was also evident (Figure 1B). The patient’s cerebrospinal fluid contained a small population of polyclonal CD19+ B cells and CD4+ and CD8+ T cells. An extensive laboratory workup, including tests for HIV, antinuclear antibodies, anti–double-stranded DNA, antineutrophil cytoplasmic antibodies, serum protein electrophoresis, erythrocyte sedimentation rate, C-reactive protein, complete blood cell count, antiganglioside antibodies, anti–myelin-associated glycoprotein antibodies, and angiotensin-converting enzyme, gave normal results. A computed tomographic (CT) scan of the thorax and a whole-body positron emission tomography (PET)/CT scan revealed no abnormalities.
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Neurosarcoidosis presenting with relapsing optic neuropathy and multiple cranial neuropathies
C. Repeat the whole-body positron emission tomography scan
Although the findings of an initial MRI of the brain were described as normal, the symptoms and clinical findings of the neuro-ophthalmologist in conjunction with the findings on review of the MRI raised suspicion for sarcoidosis involving the central nervous system. Neurosarcoidosis can present a diagnostic challenge in indolent, relapsing cases, and the clinician should maintain a high index of suspicion despite a negative workup. Given the rarity of central nervous system involvement in sarcoidosis and the low chance of false-negative results on PET, repeating the testing is essential with disease evolution as well (choice C).1,2 A repeated PET/CT scan showed fluorodeoxyglucose-avid cervical, mediastinal, and hilar lymphadenopathy in a pattern suggestive of sarcoidosis (Figure 2), and a tissue biopsy of a hilar lymph node was characterized by noncaseating granulomas with proliferation of epithelioid cells.
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Corresponding Author: Konstantinos A. A. Douglas, MD, DVM, MBA, Neuro-Ophthalmology Service, Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114 (email@example.com).
Published Online: November 7, 2019. doi:10.1001/jamaophthalmol.2019.4410
Conflict of Interest Disclosures: None reported.
Additional Information: Drs Douglas and Douglas were co–first authors.
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