Lauren M Webb1, John J Chen2, Allen J Aksamit3, Shamik Bhattacharyya4, Bart K Chwalisz5, Denis Balaban4, Giovanna S Manzano4, Ahya S Ali4, Jennifer Lord6, Stacey L Clardy6, Rohini D Samudralwar7, Yang Mao-Draayer8, James A Garrity9, M Tariq Bhatti2, Lindsey E Turner10, Eoin P Flanagan11. 1. Mayo Clinic Alix School of Medicine, 200 1st St. SW, Rochester, MN, USA. 2. Mayo Clinic, Department of Ophthalmology, 200 1st St. SW, Rochester, MN, USA; Mayo Clinic, Department of Neurology, 200 1st St. SW, Rochester, MN, USA. 3. Mayo Clinic, Department of Neurology, 200 1st St. SW, Rochester, MN, USA. 4. Brigham and Women's Hospital, Department of Neurology, 75 Francis St., Boston, MA, USA. 5. Massachusetts General Hospital, Department of Neurology, Department of Ophthalmology, and Massachusetts Eye and Ear Infirmary, 243 Charles St., Boston, MA, USA. 6. University of Utah, Department of Neurology, 50 N. Medical Dr., Salt Lake City, UT, USA. 7. University of Texas Health Science Center, Department of Neurology, 6410 Fannin St., Houston, TX, USA. 8. University of Michigan, Department of Neurology, 1500 E. Medical Center Dr., Ann Arbor, MI, USA. 9. Mayo Clinic, Department of Ophthalmology, 200 1st St. SW, Rochester, MN, USA. 10. Mayo Clinic, Graduate School of Biomedical Sciences, 200 1st St. SW, Rochester, MN, USA. 11. Mayo Clinic, Department of Neurology, 200 1st St. SW, Rochester, MN, USA. Electronic address: Flanagan.Eoin@mayo.edu.
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.
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.