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A 12-year-old girl presented for assessment of abnormal gait. A year prior, she was found to have bilateral panuveitis with occlusive retinal periphlebitis involving all quadrants of the central and peripheral retina with capillary nonperfusion (Figure 1). Her subsequent workup revealed elevated inflammatory markers and subcentimeter chest and abdominal lymph nodes. Given a positive interferon-γ release assay, therapy was initiated with rifampin, isoniazid, pyrazinamide, ethambutol (RIPE) and corticosteroids for presumed tuberculosis.
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B. Perform a lacrimal gland biopsy
A right orbitotomy with lacrimal gland biopsy was performed (choice B) allowing for histopathologic confirmation of sarcoidosis (Figure 2). Sarcoidosis is a protean multisystem inflammatory disorder classically defined histologically by well-formed, non-necrotizing, “naked” epithelioid granulomas. Focal histologic necrosis can be seen, particularly in the lacrimal gland.1 Neurosarcoidosis can manifest anywhere in the nervous system including as intramedullary spinal cord lesions.2 Neurologic manifestations occur in approximately 5% of adults with sarcoidosis and is even rarer in children.3 Fifty-five pediatric cases have been reported of which 10 presented as isolated neurosarcoidosis.4 The average age of presentation of pediatric neurosarcoidosis is 12 years and the most common manifestations are seizure, cranial neuropathies, hypothalamic dysfunction, and optic nerve swelling.4 Spinal cord involvement is unusual in children, and to our knowledge, this is the third case reported in the literature.4 Symptoms present subacutely, with varying combinations of pain, limb paraparesis, paresthesias, and bowel or bladder dysfunction.2 Cerebrospinal fluid (CSF) studies typically show a lymphocytic pleocytosis and elevated protein level.5
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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
Corresponding Author: Nahyoung G. Lee, MD, Massachusetts Eye and Ear, 243 Charles St, Boston, MA 02114 (firstname.lastname@example.org).
Published Online: December 23, 2021. doi:10.1001/jamaophthalmol.2021.3076
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank Sarkis Soukiasian, MD, Lahey Medical Center, for the fundus images. We also thank the patient’s mother for granting permission to publish this information.
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