Abstract
Headache, a common and disabling symptom in Behçet’s syndrome, may be associated with a variety of neurologic syndromes and ocular inflammation, or may present as an isolated feature. Our objective is to describe the various neurologic and ocular syndromes of Behçet’s syndrome of which headache is a symptom, and to review the features of isolated headaches in Behçet’s. We also report results of a study of headache in Behçet's syndrome patients who are followed at NYU Hospital for Joint Diseases, the first study of its kind in North American patients, and the first to document prevalence of both episodic and chronic daily headache in Behçet’s.
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Behçet H, Matteson EL. On relapsing, aphthous ulcers of the mouth, eye and genitalia caused by a virus. 1937. Clin Exp Rheumatol. 2010;28:S2–5.
Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet 1990;335:1078-1080
Hatemi G, Yazici Y, Yazici H. Behçet's syndrome. Rheum Dis Clin N Am. 2013;39:245–61. Up-to-date overview of epidemiology, symptamtology, and treatment approaches in Behcet's.
Yazici H, Fresko I, Yurdakul S. Behçet's syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol. 2007;3:148–55.
O'Duffy JD. Behçet's disease. Curr Opin Rheumatol. 1994;6:39–43.
Siva A, Saip S. The spectrum of nervous system involvement in Behçet's syndrome and its differential diagnosis. J Neurol. 2009;256:513–29.
Siva A, Altintas A, Saip S. Behçet's syndrome and the nervous system. Curr Opin Neurol. 2004;17:347–57.
Kidd D. The prevalence of headache in Behçet's syndrome. Rheumatology. 2006;45:621–3.
Evans RW, Akman-Demir G. Behçet syndrome and headache. Headache. 2004;44:102–4.
Hirohata S, Kikuchi H, Sawada T, et al. Clinical characteristics of neuro-Behçet's disease in Japan: a multicenter retrospective analysis. Mod Rheumatol Japan Rheum Assoc. 2012;22:405–13.
Gökçay F, Celebisoy N, Gökçay A, Aksu K, Keser G. Neurological symptoms and signs in Behçet disease: a Western Turkey experience. Neurologist. 2011;17:147–50.
Koçer N, Islak C, Siva A, et al. CNS involvement in neuro-Behçet syndrome: an MR study. AJNR Am J Neuroradiol. 1999;20:1015–24.
Monastero R, Camarda C, Pipia C, et al. Cognitive impairment in Behçet's disease patients without overt neurological involvement. J Neurol Sci. 2004;220:99–104.
Saip S, Akman-Demir G, Siva A. Neuro-Behçet syndrome. Handb Clin Neurol. 2014;121:1703–23.
Kikuchi S, Niino M, Shinpo K, Terae S, Tashiro K. Intracranial hemorrhage in neuro-Behçet's syndrome. Intern Med. 2002;41:692–5.
Kobayashi H, Saito Y, Kaneko Y, Ishimoto K, Ohnishi Y. [A case of neuro-Behçet's disease with hemorrhagic brain stem lesion detected by MRI]. Rinsho shinkeigaku. Clin Neurol. 1992;32:763–6.
O'Duffy JD, Goldstein NP. Neurologic involvement in seven patients with Behçet's disease. Am J Med. 1976;61:170–8.
Ashjazadeh N, Borhani Haghighi A, Samangooie S, Moosavi H. Neuro-Behçet's disease: a masquerader of multiple sclerosis. A prospective study of neurologic manifestations of Behçet's disease in 96 Iranian patients. Exp Mol Pathol. 2003;74:17–22.
Uluduz D, Kurtuncu M, Yapici Z, et al. Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology. 2011;77:1900–5. An important and rare study of pediatric manifestations of neuro-Behcet's.
Zeghidi H, Saadoun D, Bodaghi B. [Ocular manifestations in Behçet's disease]. La Rev Med Interne Fondee Soc Natl Fr Med interne. 2014;35:97–102.
Nussenblatt RB. Uveitis in Behçet's disease. Int Rev Immunol. 1997;14:67–79.
Matsuo T, Itami M, Nakagawa H, Nagayama M. The incidence and pathology of conjunctival ulceration in Behçet's syndrome. Br J Ophthalmol. 2002;86:140–3.
Headache Classification Committee of the International Headache S. The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia : Int J Headache. 2013;33:629–808. This is the beta version of the ICHD 3rd edition classification of headaches.
Saip S, Siva A, Altintas A, et al. Headache in Behçet's syndrome. Headache. 2005;45:911–9.
Stovner LJ, Andree C. Prevalence of headache in Europe: a review for the Eurolight project. J Headache Pain. 2010;11:289–99.
Lipton RB, Stewart WF, Diamond S, Diamond ML, Reed M. Prevalence and burden of migraine in the United States: data from the American Migraine Study II. Headache. 2001;41:646–57.
Schwartz BS, Stewart WF, Simon D, Lipton RB. Epidemiology of tension-type headache. JAMA : J Am Med Assoc. 1998;279:381–3.
Scher AI, Stewart WF, Liberman J, Lipton RB. Prevalence of frequent headache in a population sample. Headache. 1998;38:497–506.
Aykutlu E, Baykan B, Akman-Demir G, Topcular B, Ertas M. Headache in Behçet's disease. Cephalalgia : Int J Headache. 2006;26:180–6.
Borhani Haghighi A, Aflaki E, Ketabchi L. The prevalence and characteristics of different types of headache in patients with Behçet's disease, a case-control study. Headache. 2008;48:424–9.
Zayed H, Effat D, Nawito Z, et al. Silent central nervous system involvement in Egyptian Behçet's disease patients: clinical, psychiatric, and neuroimaging evaluation. Clin Rheumatol. 2011;30:1173–80.
Hanly JG, Urowitz MB, O'Keeffe AG, et al. Headache in systemic lupus erythematosus: results from a prospective, international inception cohort study. Arthritis Rheum. 2013;65:2887–97. Outstanding contribution to literature on headache in a common autoimmune disoder – systemic lupus erythematosis. Likely to be a definitive reference in the field.
Buse DC, Manack AN, Fanning KM, et al. Chronic migraine prevalence, disability, and sociodemographic factors: results from the American Migraine Prevalence and Prevention Study. Headache. 2012;52:1456–70.
Riera-Mestre A, Martinez-Yelamos S, Martinez-Yelamos A, Ferrer I, Pujol R, Vidaller A. Clinicopathologic features and outcomes of neuro-Behçet disease in Spain: a study of 20 patients. Eur J Intern Med. 2010;21:536–41.
Al-Araji A, Sharquie K, Al-Rawi Z. Prevalence and patterns of neurological involvement in Behçet's disease: a prospective study from Iraq. J Neurol Neurosurg Psychiatry. 2003;74:608–13.
Dodick DW. Chronic daily headache. N Engl J Med. 2006;354(2):158–65.
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Conflict of Interest
Dr. Vijay Vishwanath, Dr. Ericka Wong, Dr. Sara Crystal, Dr. Maria Filopoulos, and Dr. Ilya Kister each declare no potential conflict of interest.
Dr. Matthew S. Robbins is a section editor for Current Pain and Headache Reports. Dr. Robbins has received honoraria from Hofstra North Shore-LIJ School of Medicine and SUNY Downstate College of Medicine, and payment for manuscript preparation from MedLink Neurology and the American College of Physicians (PIER Module). Dr. Robbins has received payment for development of educational presentations, including service on speakers' bureaus, from the American Headache Society and Prova Education CME, and royalties from Headache.
Dr. Richard B Lipton is a consultant for Allergan, the American Headache Society, Autonomic Technologies, Boston Scientific, CogniMed, CoLucid, Eli Lilly, eNeura Therapeutics, Merck, Novartis, NuPathe, Vedanta, Zogenix, Alder, Bristol-Myers Squibb, ElectroCore, GlaxoSmithKline, and Nautilus. Dr. Lipton reports a grant and payment for manuscript preparation from NIH. Dr. Lipton has given expert testimony for Novartis Pharmaceuticals and has received payment for lectures from Allergan. Dr. Lipton has stock in eNeura and has had travel/accommodation expense covered by the American Headache Society.
Dr. Yusuf Yazici is a consultant for AbbVie, Celgene, BMS, Genentech, Pfizer, UCB, and Samumed, and reports grants from Celgene, BMS, and Genentech.
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Vijay Vishwanath and Ericka Wong contributed equally to this work.
This article is part of the Topical Collection on Secondary Headache
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Vishwanath, V., Wong, E., Crystal, S.C. et al. Headache in Behçet’s Syndrome: Review of Literature and NYU Behçet’s Syndrome Center Experience. Curr Pain Headache Rep 18, 445 (2014). https://doi.org/10.1007/s11916-014-0445-0
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DOI: https://doi.org/10.1007/s11916-014-0445-0