title: Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations.
abstract: Neuro-Behçet's disease (NBD) is one of the more serious manifestations of Behçet's disease (BD), which is a relapsing inflammatory multisystem disease with an interesting epidemiology. Though NBD is relatively uncommon, being potentially treatable, neurologists need to consider it in the differential diagnosis of inflammatory, infective, or demyelinating CNS disorders. Evidence-based information on key issues of NBD diagnosis and management is scarce, and planning for such studies is challenging. We therefore initiated this project to develop expert consensus recommendations that might be helpful to neurologists and other clinicians, created through an extensive literature review and wide consultations with an international advisory panel, followed by a Delphi exercise. We agreed on consensus criteria for the diagnosis of NBD with two levels of certainty in addition to recommendations on when to consider NBD in a neurological patient, and on the use of various paraclinical tests. The management recommendations included treatment of the parenchymal NBD and cerebral venous thrombosis, the use of disease modifying therapies, prognostic factors, outcome measures, and headache in BD. Future studies are needed to validate the proposed criteria and provide evidence-based treatments.
authors.affiliation: Neurology Research Department, University Hospital of North Staffordshire, Stoke-on-Trent, England, UK.
authors.forename: Seema
authors.initials: S
authors.lastname: Kalra
authors.forename: Alan
authors.initials: A
authors.lastname: Silman
authors.forename: Gulsen
authors.initials: G
authors.lastname: Akman-Demir
authors.forename: Saeed
authors.initials: S
authors.lastname: Bohlega
authors.forename: Afshin
authors.initials: A
authors.lastname: Borhani-Haghighi
authors.forename: Cris S
authors.initials: CS
authors.lastname: Constantinescu
authors.forename: Habib
authors.initials: H
authors.lastname: Houman
authors.forename: Alfred
authors.initials: A
authors.lastname: Mahr
authors.forename: Carlos
authors.initials: C
authors.lastname: Salvarani
authors.forename: Petros P
authors.initials: PP
authors.lastname: Sfikakis
authors.forename: Aksel
authors.initials: A
authors.lastname: Siva
authors.forename: Adnan
authors.initials: A
authors.lastname: Al-Araji
mesh_terms: D001528:Behcet Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D003937:Diagnosis, Differential; D055317:Evidence-Based Practice / Q000639:trends; D006801:Humans; D020767:Intracranial Thrombosis / Q000209:etiology / Q000628:therapy; D020246:Venous Thrombosis / Q000209:etiology / Q000628:therapy