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Neuro-Ophthalmic Manifestations of Neurosarcoidosis
1Barrow Neurological Institute, Phoenix, AZ.
Abstract:
Sarcoidosis is a systemic granulomatous disorder characterized by noncaseating granulomas. This review focuses on the pathophysiology, clinical spectrum, diagnostic strategies, and management of neuro-ophthalmic sarcoidosis. The pathogenesis involves immune dysregulation in genetically predisposed individuals exposed to environmental antigens, with granuloma formation as the hallmark. Neuro-ophthalmic involvement is classified into afferent, efferent, and general neurological categories. Afferent presentations include optic neuropathy, optic neuritis, chiasmopathy, papilledema, and visual field defects among others, while efferent signs include cranial neuropathies, ophthalmoplegia, dorsal midbrain syndrome, and more. Diagnostic evaluation includes MRI with contrast, cerebrospinal fluid analysis, chest imaging, and, where possible, tissue biopsy. Management primarily involves corticosteroids, with immunosuppressive and biological agents reserved for refractory cases. Early diagnosis and treatment initiation are crucial to preserve visual function and reduce relapse. Advances in biomarker discovery and imaging modalities hold promise for more accurate and earlier diagnosis. Given its protean manifestations and potential for vision-threatening complications, sarcoidosis remains a critical consideration in the differential diagnosis of unexplained neuro-ophthalmic symptoms.
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