{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T07:38:39Z","timestamp":1773387519670,"version":"3.50.1"},"reference-count":21,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2022,9,7]],"date-time":"2022-09-07T00:00:00Z","timestamp":1662508800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Acute idiopathic blind spot enlargement syndrome (AIBSES) is characterized by unilateral visual field loss in the blind spot area, acute onset photopsia, and funduscopically few or no optic disc changes. AIBSES predominantly affects young adults and is often misdiagnosed as optic neuritis because of low awareness. Optical coherence tomography (OCT) has become the gold standard in diagnosing AIBSES as a disease of the outer retina. In our case series, we present three consecutive patients with AIBSES followed prospectively with and without steroid therapy. The patients, aged 25 to 27 years, presented in our neuroophthalmology department between 2020 and 2021. We report their disease course and management and discuss therapeutic options, as no well-established procedures exist. Common pitfalls and diagnostic errors are analysed. Two women and one man showed unilateral acute-onset photopsia and blind spot enlargement on perimetry without visual acuity reduction. Spectral domain OCT (Heidelberg Engineering, Heidelberg, Germany) revealed marked peripapillary changes in the ellipsoid zone and autofluorescence in all patients, corresponding to faint blurring of the optic disc margin. Characteristically, there was no P100 latency delay in the visual evoked potential in any of the patients. Two patients received weight-adapted oral prednisolone, which was gradually tapered over six to eight weeks. Two patients showed full recovery of their symptoms at six and seven months after onset, while mild defect healing was seen in one treated patient after 12 months. Follow-up OCT showed restoration of the outer retinal layers 6\u201312 months after disease onset. Careful history taking and an unprejudiced ophthalmological workup helps in diagnosing AIBSES in young adults with unilateral acute visual field defects. While its etiology is still unclear, accurate diagnosis of AIBSES can be made with peripapillary OCT. In our cases, the disease course of AIBSES was much better than its reputation. Early corticosteroid treatment may support outer retinal reorganisation, which can be followed with OCT in accordance with visual field restoration. This should be addressed in a prospective study.<\/jats:p>","DOI":"10.3390\/jcm11185278","type":"journal-article","created":{"date-parts":[[2022,9,8]],"date-time":"2022-09-08T01:40:13Z","timestamp":1662601213000},"page":"5278","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Acute Idiopathic Blind Spot Enlargement Syndrome\u2014New Perspectives in the OCT Era"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0007-147X","authenticated-orcid":false,"given":"Julian A.","family":"Zimmermann","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, University of Muenster Medical Center, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicole","family":"Eter","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University of Muenster Medical Center, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5510-3857","authenticated-orcid":false,"given":"Julia","family":"Biermann","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University of Muenster Medical Center, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,9,7]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1001\/archopht.1988.01060130050026","article-title":"Acute idiopathic blind spot enlargement. A big blind spot syndrome without optic disc edema","volume":"106","author":"Fletcher","year":"1988","journal-title":"Arch. Ophthalmol."},{"key":"ref_2","first-page":"79","article-title":"Acute zonal occult outer retinopathy. Donders Lecture: The Netherlands Ophthalmological Society, Maastricht, Holland, June 19, 1992","volume":"13","author":"Gass","year":"1993","journal-title":"J. Clin. Neuroophthalmol."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Krieglstein, G.K., Weinreb, R.N., Pleyer, U., and Mondino, B. (2005). Primary Inflammatory Choriocapillaropathies. Uveitis and Immunological Disorders, Springer.","DOI":"10.1007\/b138026"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1097\/00006982-199515050-00001","article-title":"MEWDS, MFC, PIC, AMN, AIBSE, and AZOOR: One disease or many?","volume":"15","author":"Jampol","year":"1995","journal-title":"Retina"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"100949","DOI":"10.1016\/j.ajoc.2020.100949","article-title":"Acute idiopathic blind spot enlargement syndrome following influenza vaccination","volume":"20","author":"Quinones","year":"2020","journal-title":"Am. J. Ophthalmol. Case Rep."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"e199","DOI":"10.1016\/j.jcjo.2018.09.005","article-title":"Acute idiopathic blind spot enlargement syndrome following measles, mumps and rubella vaccination","volume":"54","author":"Wong","year":"2019","journal-title":"Can. J. Ophthalmol."},{"key":"ref_7","first-page":"59","article-title":"Acute idiopathic blind spot enlargement syndrome: A review of 27 new cases","volume":"119","author":"Volpe","year":"2001","journal-title":"Arch. Ophthalmol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1001\/jamaophthalmol.2017.5470","article-title":"Incidence and Causes of Overdiagnosis of Optic Neuritis","volume":"136","author":"Stunkel","year":"2017","journal-title":"JAMA Ophthalmol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1212\/WNL.0000000000002318","article-title":"Overdiagnosis of idiopathic intracranial hypertension","volume":"86","author":"Fisayo","year":"2016","journal-title":"Neurology"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1001\/archopht.1988.01060140207023","article-title":"The Optic Neuritis Treatment Trial","volume":"106","author":"Beck","year":"1988","journal-title":"Arch. Ophthalmol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1016\/S0161-6420(91)32266-8","article-title":"Acute Idiopathic Blind Spot Enlargement","volume":"98","author":"Singh","year":"1991","journal-title":"Ophthalmology"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1001\/archopht.1991.01080120052024","article-title":"Optic neuritis and ischemic optic neuropathy. Overlapping clinical profiles","volume":"109","author":"Rizzo","year":"1991","journal-title":"Arch. Ophthalmol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1326","DOI":"10.1016\/S0161-6420(02)01066-7","article-title":"Clinical features and natural history of the acute idiopathic enlarged blind spot syndrome","volume":"109","author":"Watzke","year":"2002","journal-title":"Ophthalmology"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1159\/000327431","article-title":"Outer retinal microstructure in a case of acute idiopathic blind spot enlargement syndrome","volume":"2","author":"Sugahara","year":"2011","journal-title":"Case Rep. Ophthalmol."},{"key":"ref_15","first-page":"1","article-title":"Acute Idiopathic Blind Spot Enlargement Syndrome in A 63-Year-Old Man","volume":"11","author":"Makino","year":"2014","journal-title":"Internet J. Ophthalmol. Vis. Sci."},{"key":"ref_16","first-page":"143","article-title":"Clinical features and differential diagnosis of acute idiopathic blind spot enlargement syndrome","volume":"29","author":"Liu","year":"2014","journal-title":"Eye Sci."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"494","DOI":"10.3109\/09273948.2013.872802","article-title":"High-definition optical coherence tomography findings in acute idiopathic blind spot enlargement (AIBSE) syndrome","volume":"22","author":"Piri","year":"2014","journal-title":"Ocul. Immunol. Inflamm."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.ajoc.2016.02.002","article-title":"Recovery of outer retina in acute idiopathic blind spot enlargement (AIBSE)","volume":"1","author":"Trese","year":"2016","journal-title":"Am. J. Ophthalmol. Case Rep."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1016\/S0002-9394(02)01640-9","article-title":"Acute zonal occult outer retinopathy: A long-term follow-up study","volume":"134","author":"Gass","year":"2002","journal-title":"Am. J. Ophthalmol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1080\/01658107.2017.1400076","article-title":"From A\u2026 to\u2026 Z(OOR): The Clinical Spectrum of Acute Zonal Occult Outer Retinopathy","volume":"42","author":"Wang","year":"2018","journal-title":"Neuro Ophthalmol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1159\/000456724","article-title":"Fundus Autofluorescence Imaging in the Assessment of Acute Zonal Occult Outer Retinopathy","volume":"237","author":"Wang","year":"2017","journal-title":"Ophthalmologica"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/18\/5278\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:24:54Z","timestamp":1760142294000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/18\/5278"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,9,7]]},"references-count":21,"journal-issue":{"issue":"18","published-online":{"date-parts":[[2022,9]]}},"alternative-id":["jcm11185278"],"URL":"https:\/\/doi.org\/10.3390\/jcm11185278","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,9,7]]}}}