Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.
ADVANCED SEARCH
Log in
Top
Journal of Neurology
Published in: Journal of Neurology 12/2013

01-12-2013 | Original Communication

Optic neuropathy in Behçet’s syndrome

Author: Desmond P. Kidd

Published in: Journal of Neurology | Issue 12/2013

Login to get access

Abstract

This paper presents the clinical features, investigation results and response to treatment of four cases of primary optic neuropathy in Behçet’s syndrome, with a comprehensive literature search for all other published cases. A total of 20 cases has been identified; the clinical syndrome is a unilateral or bilateral optic neuropathy of inflammatory type in which pain arises in 20 % and optic disc swelling seen in 45 % of cases. The condition was relapsing in four cases, and a progressive optic neuropathy was seen in one case. All but the progressive case responded to steroids with or without additional immunosuppression. The nadir visual acuity was the only determinant of visual outcome. 67 % of cases reported a return to normal acuity following treatment.
Literature
1.
Yazici H, Fresko I, Yurdakul S (2007) Behçet’s syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol 3:148-55PubMedCrossRef
2.
Deuter CM, Kotter I, Wallace GR, Murray PI, Stubiger N, Zierhut M (2008) Behçet’s disease: ocular effects and treatment. Prog Retin Eye Res 27:111–136PubMedCrossRef
3.
Azizlerli G, Kose AA, Sarica R, Gul A, Tutkin IT, Kulac M, Tunc R, Urgancioglu M, Disci R (2003) Prevalence of Behçet’s disease in Istanbul Turkey. Int J Dermatol 42:803–806PubMedCrossRef
4.
Yamamoto SI, Toykawa H, Matsubara J et al (1974) A nationwide survey of Behçet’s disease in Japan. I. Epidemiological survey. Jpn J Ophthalmol 18:282–290
5.
Kidd D (2003) The prevalence of Behçet’s syndrome and its neurological complications in Hertfordshire UK. Adv Exp Med Biol 528:95–97PubMedCrossRef
6.
Al Araji A, Kidd D (2009) Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol 8:192–204PubMedCrossRef
7.
Yesilot N, Bahar S, Yilmazer S, Mutlu M, Kurtuncu M, Tuncay R, Coban O, Akman Demir G Cerebral venous sinus thrombosis in Behçet’s disease compared to those associated with other etiologies. J Neurol in press (epub 12 March 2009)
8.
Tugal Tutkin I, Onal S, Altan-Yaycioglu R, Huseyin Altunbas H, Urgancioglu M (2004) Uveitis in Behçet disease: an analysis of 880 patients. Am J Ophthalmol 138:373–380CrossRef
9.
Akman-Demir G, Serdaroglu P, Tasci B (1999) Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. The Neuro-Behçet study group. Brain 122:2171–2182PubMedCrossRef
10.
Kidd D, Steuer A, Denman M, Rudge P (1999) Neurological complications in Behçet’s syndrome. Brain 122:2183–2194PubMedCrossRef
11.
Al Fahad SA, Al Araji AH (1999) Neuro-Behcet’s disease in Iraq: a study of 40 patients. J Neurol Sci 170:105–111PubMedCrossRef
12.
Siva A, Kantarci OH, Saip S, Altintas A, Hamuryudan V, Islak C, Kocer N, Yazici H (2001) Behçet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 248:95–103PubMedCrossRef
13.
Colvard DM, Robertson DM, O’Duffy JD (1977) The ocular manifestations of Behçet’s disease. Arch Ophthalmol 95:1813–1817PubMedCrossRef
14.
Scouras J, Koutroumanos J (1976) Ischaemic optic neuropathy in Behçet’s syndrome. Ophthalmologica 173:11–18PubMedCrossRef
15.
Kansu T, Kirkali P, Kansu E, Zileli T (1989) Optic neuropathy in Behçet’s disease. J Clin Neuro-ophthalmol 9:277–280
16.
Salvi F, Mascalchi M, Malatesta R, Olivieri I, Molinotti C, Capaccioli L, Testoni S, Tassinari CA (1999) Optic neuropathy in Behçet’s disease: report of two cases. Ital J Neurol Sci 20:183–186PubMedCrossRef
17.
Mitra S, Koul RL (1999) Paediatric Neuro-Behcet’s disease presenting with optic nerve head swelling. Br J Ophthalmol 83:1096PubMedCrossRef
18.
19.
Nakamura T, Takahashi K, Kishi S (2002) Optic nerve involvement in Neuro-Behçet’s disease. Jpn J Ophthalmol 46:100–102PubMedCrossRef
20.
Yalcindag N, Yilmaz N, Takeli O, Ozdemir O (2004) Acute optic neuropathy in Behçet disease. Eur J Ophthalmol 14:578–580PubMed
21.
Tarzi MD, Lightman S, Longhurst HJ (2005) An exacerbation of Behçet’s syndrome presenting with bilateral papillitis. Rheumatology 44:953–954PubMedCrossRef
22.
Yamauchi Y, Cruz JM, Kaplan HJ, Goto H, Sakai J, Usui M (2005) Suspected simultaneous bilateral anterior ischemic optic neuropathy in a patient with Behçet’s disease. Ocul Immunol Inflamm 13:317–325PubMedCrossRef
23.
Voros GM, Sandhu SS, Pandit R (2006) Acute optic neuropathy in patients with Behçet’s disease. Ophthalmologica 220:400–405PubMedCrossRef
24.
Nanke Y, Kotake S, Goto M, Matsubara M, Jihara H (2009) A Japanese case of Behçet’s disease complicated by recurrent optic neuropathy involving both eyes: a third case in the English literature. Mod Rheumatol 19:334–337PubMedCrossRef
25.
Çetin E, Yaylah V, Yildirim C (2011) Isolated optic neuropathy in a case of Behçet’s disease. Int Ophthalmol 31:153–155PubMedCrossRef
26.
Optic neuritis study group (1991) The clinical profile of optic neuritis. Experience of the optic neuritis study group. Arch Ophthalmol 109:1673–1678CrossRef
27.
Gallinaro C, Robinet-Combes A, Sale Y, Richard P, Saraux A, Colin J (1995) Neuropapillite de la maladie de Behcet Une observation. J Fr d’Ophtalmol 18:147–150
28.
Lamari H, Baha AT, Benhaddou M, Alikane O, Hamdani M, Zaghloul K, Amraoui A (2003) Les atteintes du nerf optique au cours de la maladie de Behçet (a propos de 148 cas). Bull Soc Belg Ophtalmol 289:9–14
29.
Joseph FG, Scolding NJ (2007) Neuro-Behçet’s disease in Caucasians: a study of 22 patients. Eur J Neurol 14:174-80PubMedCrossRef
30.
Uga S, Ishikura S, Wakakura M (1977) Histopathology of the optic nerve and retina in Behçet’s disease. In: Dilsen N, Konice M, Ovul C (eds) Proceedings of an international symposium on Behçet’s disease. Amsterdam; Exerpta Medica pp 85–87
31.
Chavis PS, Tabbara KF (1998) Demyelination of retinal myelinated fibres in Behçet’s disease. Doc Ophthalmol 95:157–164PubMedCrossRef
Metadata
Title
Optic neuropathy in Behçet’s syndrome
Author
Desmond P. Kidd
Publication date
01-12-2013
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 12/2013
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-013-7070-4

Other articles of this Issue 12/2013

Journal of Neurology 12/2013 Go to the issue

Erratum

Erratum to: R208H-129VV haplotype in the prion protein gene: phenotype and neuroimaging of a patient with genetic Creutzfeldt-Jakob disease

Original Communication

Intravenous amantadine on freezing of gait in Parkinson’s disease: a randomized controlled trial

Letter to the Editors

Aquaporin-4 antibody neuromyelitis optica following anti-NMDA receptor encephalitis

Original Communication

Levels of HVA, 5-HIAA, and MHPG in the CSF of vascular parkinsonism compared to Parkinson’s disease and controls

Original Communication

Prominent psychiatric symptoms in patients with Parkinson’s disease and concomitant argyrophilic grain disease

Original Communication

Results of the PERFORM magnetic resonance imaging study