@article{157c29906565423886f320a773d87715,
title = "A multi-center case series of sarcoid optic neuropathy",
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.",
keywords = "Optic neuropathy, Sarcoidosis, Vision",
author = "Webb, {Lauren M.} and Chen, {John J.} and Aksamit, {Allen J.} and Shamik Bhattacharyya and Chwalisz, {Bart K.} and Denis Balaban and Manzano, {Giovanna S.} and Ali, {Ahya S.} and Jennifer Lord and Clardy, {Stacey L.} and Samudralwar, {Rohini D.} and Yang Mao-Draayer and Garrity, {James A.} and Bhatti, {M. Tariq} and Turner, {Lindsey E.} and Flanagan, {Eoin P.}",
note = "Funding Information: Ms. Webb reports no disclosures; Dr. Chen reports no disclosures; Dr. Aksamit reports no disclosures; Dr. Bhattacharyya has received consulting fees from Teladoc and Alexion Pharmaceuticals and publishing honorarium from Springer, UpToDate, and American Academy of Neurology; Dr. Chwalisz reports no disclosures; Dr. Balaban reports no disclosures; Dr. Manzano reports no disclosures; Dr. Ali reports no disclosures; Dr. Lord reports no disclosures; Dr. Clardy reports no disclosures; Dr. Samudralwar reports no disclosures; Dr. Mao-Draayer has served as a consultant and/or received grant support from from Acorda, Bayer Pharmaceutical, Biogen Idec , Chugai Pharmaceutical , EMD Serono , Genzyme-Sanofi, Novartis, Questor, Genentech, and Teva Neuroscience. YMD is currently supported by grants from NIH NIAID Autoimmune Center of Excellence UM1-AI144298-01, CMSC, PCORI, Genzyme-Sanofi, Novartis, and Genentech; Dr. Garrity reports no disclosures; Dr. Bhatti reports no disclosures; Ms. Turner reports no disclosures; Dr. Flanagan is a site principal investigator in a randomized placebo-controlled clinical trial of inebilizumab (a CD19 inhibitor) in neuromyelitis optica spectrum disorders funded by MedImmune/Viela Bio. He receives no personal compensation and just receives reimbursement for the research activities related to the trial. Funding Information: Ms. Webb reports no disclosures; Dr. Chen reports no disclosures; Dr. Aksamit reports no disclosures; Dr. Bhattacharyya has received consulting fees from Teladoc and Alexion Pharmaceuticals and publishing honorarium from Springer, UpToDate, and American Academy of Neurology; Dr. Chwalisz reports no disclosures; Dr. Balaban reports no disclosures; Dr. Manzano reports no disclosures; Dr. Ali reports no disclosures; Dr. Lord reports no disclosures; Dr. Clardy reports no disclosures; Dr. Samudralwar reports no disclosures; Dr. Mao-Draayer has served as a consultant and/or received grant support from from Acorda, Bayer Pharmaceutical, Biogen Idec, Chugai Pharmaceutical, EMD Serono, Genzyme-Sanofi, Novartis, Questor, Genentech, and Teva Neuroscience. YMD is currently supported by grants from NIH NIAID Autoimmune Center of Excellence UM1-AI144298-01, CMSC, PCORI, Genzyme-Sanofi, Novartis, and Genentech; Dr. Garrity reports no disclosures; Dr. Bhatti reports no disclosures; Ms. Turner reports no disclosures; Dr. Flanagan is a site principal investigator in a randomized placebo-controlled clinical trial of inebilizumab (a CD19 inhibitor) in neuromyelitis optica spectrum disorders funded by MedImmune/Viela Bio. He receives no personal compensation and just receives reimbursement for the research activities related to the trial. Publisher Copyright: {\textcopyright} 2020 Elsevier B.V.",
year = "2021",
month = jan,
day = "15",
doi = "10.1016/j.jns.2020.117282",
language = "English (US)",
volume = "420",
journal = "Journal of the Neurological Sciences",
issn = "0022-510X",
publisher = "Elsevier",
}