Abstract
Neuro-Behcet’s disease (NBD) is one of the more serious manifestations of Behcet’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.
Highlights
Hulusi Behcet, a Turkish dermatologist, described the triad of recurrent oral and genital ulcers with uveitis in 1937 [1]; the disease is commonly referred to as Behcet’s disease (BD) and is recognized as a multisystem inflammatory disorder of unknown aetiology [2]
The disease terminology was voted and agreed upon as ’neuro-Behcet’s disease’, as two-thirds of the advisory group members preferred to use this term to describe the neurological manifestations of BD (66 % voted for neuroBehcet’s disease, 22 % for neuro-Behcet syndrome, and 12 % for either/other)
The following section covers the consensus recommendation with a brief summary of the relevant literature review divided into the following subsections:
Summary
Hulusi Behcet, a Turkish dermatologist, described the triad of recurrent oral and genital ulcers with uveitis in 1937 [1]; the disease is commonly referred to as Behcet’s disease (BD) and is recognized as a multisystem inflammatory disorder of unknown aetiology [2]. BD is more prevalent along the ancient Silk Road, including countries in the Far East, the Middle East, and the Mediterranean basin [3]. It has been reported, from most countries across the globe [4]. The various systemic features of BD and its commonly accepted diagnostic criteria, in addition to the description of the various epidemiological and clinical features of NBD have been described in previous publications [3,4,5]. Published studies include mainly personal experiences or single-centre approaches
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