Neuro-Ophthalmic Sarcoidosis

Giovanni Campagna, Claudia M. Prospero Ponce, Aroucha Vickers, Bennett Yau Bun Hong, Francesco Pellegrini, Daniele Cirone, Francesco Romano, Piernicola Machin, Andrew G. Lee

Research output: Contribution to journalArticle

Abstract

Background: In the absence of confirmatory biopsy, the criteria for diagnosis of neuro-ophthalmic sarcoidosis are not well established. Diagnostic criteria for both intraocular sarcoidosis and neurosarcoidosis have been proposed, but the diagnosis of neuro-ophthalmic sarcoidosis remains challenging. It is our intention to augment what is currently known about the diagnosis of neuro-ophthalmic sarcoidosis by providing a series of biopsy-proven cases that contribute to the continued development of diagnostic criteria for this enigmatic condition. Methods: Case series of four Caucasian women with biopsy-proven neuro-ophthalmic sarcoidosis. Results: The first patient was initially diagnosed with traumatic optic neuropathy following a fall. Years later, the presence of pathologic submandibular lymphadenopathy was identified and biopsied, revealing non-caseating granulomas. The second and third cases involved sarcoidosis of the extraocular muscles without clear or common systemic features of sarcoidosis. In the fourth and final case, the patient presented with a Horner syndrome attributed to sarcoid infiltration of the ipsilateral sympathetic chain. Bronchoscopy with biopsy showed non-caseating granulomas consistent with sarcoidosis. Conclusions: We describe four cases of neuro-ophthalmic sarcoidosis and propose possible neuro-orbital and neuro-ophthalmic criteria both with and without diagnostic biopsy.

Original languageEnglish (US)
Pages (from-to)319-326
Number of pages8
JournalNeuro-Ophthalmology
Volume44
Issue number5
DOIs
StatePublished - Feb 26 2019

Keywords

  • Neuro-ophthalmic sarcoidosis
  • diagnostic criteria
  • non-caseating granuloma
  • sarcoidosis

ASJC Scopus subject areas

  • Ophthalmology
  • Clinical Neurology

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