A multi-center case series of sarcoid optic neuropathy

Lauren M. Webb, John J. Chen, Allen J. Aksamit, Shamik Bhattacharyya, Bart K. Chwalisz, Denis Balaban, Giovanna S. Manzano, Ahya S. Ali, Jennifer Lord, Stacey L. Clardy, Rohini D. Samudralwar, Yang Mao-Draayer, James A. Garrity, M. Tariq Bhatti, Lindsey E. Turner, Eoin P. Flanagan

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: The diagnosis of sarcoid optic neuropathy is time-sensitive, as delayed treatment risks irreversible vision loss. We sought to analyze its characteristics and outcomes. Methods: We performed a multi-center retrospective study of sarcoid optic neuropathy among 5 USA medical centers. Inclusion criteria were: 1) clinical optic neuropathy; 2) optic nerve/sheath enhancement on neuroimaging; 3) pathological confirmation of systemic or nervous system sarcoidosis. Results: Fifty-one patients were included. The median onset age of sarcoid optic neuropathy was 50 years (range, 17–70 years) and 71% were female. The median visual acuity at nadir in the most affected eye was 20/80 (range, 20/20 to no-light-perception). Thirty-four of 50 (68%) patients had radiologic evidence of other nervous system involvement and 20 (39%) patients had symptoms/signs of other cranial nerve dysfunction. Cerebrospinal fluid analysis revealed an elevated white blood cell count in 22 of 31 (71%) patients (median: 14/μL; range: 1–643/μL). Pathologic confirmation of sarcoidosis was by biopsy of systemic/pulmonary site, 34 (67%); optic nerve/sheath, 9 (18%); or other nervous system region, 8 (16%). Forty patients improved with treatment (78%), 98% receiving corticosteroids and 65% receiving steroid-sparing immunosuppressants, yet 11/46 patients (24%) had a visual acuity of 20/200 or worse at last follow-up. Conclusions: Sarcoid optic neuropathy frequently occurs with other clinical and radiologic abnormalities caused by neurosarcoidosis and diagnostic confirmation occasionally requires optic nerve/sheath biopsy. Improvement with treatment is common but most patients have some residual visual disability. Improved recognition and a more expeditious diagnosis and treatment may spare patients from permanent vision loss.

Original languageEnglish (US)
Article number117282
JournalJournal of the neurological sciences
Volume420
DOIs
StatePublished - Jan 15 2021

Keywords

  • Optic neuropathy
  • Sarcoidosis
  • Vision

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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