Headache in Behçet’s Syndrome: Review of Literature and NYU Behçet’s Syndrome Center Experience

Vishwanath, Vijay; Wong, Ericka; Crystal, Sara C.; Robbins, Matthew S.; Filopoulos, Maria T.; Lipton, Richard B.; Yazıcı, Yusuf; Kister, Ilya

doi:10.1007/s11916-014-0445-0

Cited by 10 publications

(7 citation statements)

References 31 publications

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“…Papilledema and headaches were the only neurological symptoms significantly associated with confirmed BD. Finally, we agreed to consider neurological symptoms as a whole but without headaches, which are not specific enough in BD 22. Our PEDBD classification does not consider a positive pathergy test result and does not mention oral aphthosis as a mandatory criteria.…”

Section: Discussionmentioning

confidence: 99%

Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD

Koné‐Paut¹,

Shahram²,

Darce-Bello³

et al. 2015

Ann Rheum Dis

186

175

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BACKGROUND: We aimed to describe the main features of Behçet's disease (BD) in children in the largest prospective cohort to date and to propose a classification.\ud METHODS: An international expert consensus group was formed to define a data set of minimal symptoms for the inclusion of patients. Patients were entered prospectively during 66 months. Experts classified patients on a consensus basis. The concordance of two international classifications was analysed in confirmed patients with BD. Comparisons of subgroups of patients helped define consensus criteria. BD-associated clinical manifestations were also investigated in three control diseases extracted from an independent data set (Eurofever).\ud FINDINGS: In total, 42 centres from 12 countries included 230 patients; data for 219 (M/F ratio=1) could be analysed. The experts classified 156 patients (71.2%) as having confirmed BD. Males more often than females showed cutaneous, ocular and vascular symptoms and females more often genital aphthosis. Age at disease onset and skin and vascular involvement were lower for European than non-European children. Oral aphthosis was the presenting sign for 81% (179/219) of patients. The mean delay to the second symptom was 2.9±2.2 years. International classifications were not concordant with the expert classification. Our paediatric classification contains six categories, a minimum of three signs (each in a distinct category) defining paediatric BD. Three clinical signs discriminated our cohort from the Eurofever cohorts.\ud INTERPRETATION: We present a comprehensive description of a large cohort of patients from both European and non-European countries and propose the first classification of paediatric BD for future therapeutic trials

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Section: Discussionmentioning

confidence: 99%

Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD

Koné‐Paut¹,

Shahram²,

Darce-Bello³

et al. 2015

Ann Rheum Dis

186

175

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“…Neurologic involvement is newly considered in the definition of BD and occurs in 5.3 to 59 % of adult cases [ 50 ]. Chronic headaches are frequent in patients with BD, and may be either isolated or associated with severe NBD involvement [ 51 ]. In the PEDBD study, although not considered as a classification criterion, the presence of headaches was significantly associated with BD confirmation by the experts ( p = 0.0063) [ 13 ].…”

Section: Clinical Symptomsmentioning

confidence: 99%

Behçet’s disease in children, an overview

2016

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SummaryBD is a systemic inflammatory disease with a variable vasculitis. Paediatric onset is very rare and carries a strong genetic component. Oral ulcers and fever of unknown origin are frequent at onset and difficult to distinguish from other inflammatory disorders; therefore, expert opinion is still mandatory to recognize the disease early. An international expert consensus has recently proposed new classification criteria for children with BD. The clinical spectrum of BD is heterogeneous and influenced by gender, ethnicity and country of residence. Young males have the worst prognosis with significantly more frequent neurological, ocular and vascular involvement. BD treatment is aimed at alleviating inflammation. Among all drugs, TNFα inhibitors have become a standard to control severe ocular, neurological and digestive system involvement.

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“…However, patients should be comprehensively evaluated with MRI and LP if they develop any atypical or new headaches to look for inflammatory or thrombotic etiologies. 130 Neurologic examination should include ophthalmic evaluation since optic nerve swelling is observed in some patients with NBD. 131 About 10% of patients have "nonstructural headache of Behçet's," which is a headache that does not meet criteria for other primary headache and also is not caused by an abnormality on imaging or exam.…”

Section: Immunoglobulin G4-related Diseasementioning

confidence: 99%

Acute Neurologic Manifestations of Systemic Immune-Mediated Diseases

Galetta

Bhattacharyya

2021

Semin Neurol

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Systemic autoimmune diseases can affect the peripheral and central nervous system. In this review, we outline the common inpatient consultations for patients with neurological symptoms from rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus, sarcoidosis, immunoglobulin G4–related disease, Behçet's disease, giant cell arteritis, granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis, polyarteritis nodosa, and ankylosing spondylitis. We discuss the symptoms, diagnostic strategies, and treatment options.

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Headache in Behçet’s Syndrome: Review of Literature and NYU Behçet’s Syndrome Center Experience

Cited by 10 publications

References 31 publications

Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD

Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD

Behçet’s disease in children, an overview

Acute Neurologic Manifestations of Systemic Immune-Mediated Diseases

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