Outcomes in Neuro-Behçet's

Kevin Deane, MD


June 19, 2014

Long-term Outcome of Neuro-Behçet's Disease

Noel N, Bernard R, Wechsler B, et al
Arthritis Rheumatol. 2014;66:1306-1314

Study Summary

Behçet disease (BD) is a rare form of vasculitis largely affecting men in the Mediterranean, Middle East, and Far East, with some association with the HLA B51 allele.[1] The primary clinical manifestations of BD are oral and genital ulcerations and uveitis. Articular, gut, and large vessel involvement are possible, as well as arterial and venous thromboses. One of the most feared complications of BD is neurologic disease, with manifestations including peripheral neuropathy and brain venous thrombosis as well as inflammatory central nervous system (CNS) parenchymal disease that can manifest as headaches, strokes, encephalopathy, myelitis, and pseudotumors.[2]

Noel and colleagues presented data from a retrospective case series of 115 patients identified in France between 1974 and 2010 with CNS neuro-BD (excluding cerebral venous thrombosis). The patients were predominately men (57%), with a mean age of neuro-BD onset of 37 years. The men were from Europe (45%) and North Africa (40%), and 49% were positive for the HLA B51 antigen (method of testing not described). In terms of general BD manifestations, all patients had oral aphthous ulcers, and more than 60% also had genital ulcers and inflammatory eye disease. The most common neurologic manifestations were headaches (62%) and hemiparesis (51%), with other manifestations including sensory symptoms, ataxia, confusion, coma, and seizures affecting up to 20% of patients. Brain MRI was abnormal in more than 70% of patients, with abnormalities most commonly seen in the brainstem region. Only 14% had isolated supratentorial lesions, and 3% had MRI-defined myelitis. Cerebrospinal fluid abnormalities were seen in 59% of patients, consisting primarily of elevated cell counts.

At least 1 flare of neuro-BD was experienced by 33% of the patients during a median follow-up of 73 months. Factors associated with worse outcomes and/or increased risk for flares included coma at onset, pyramidal signs, and the presence of the HLA B51 antigen. Of note, patients who received a combination of corticosteroids and cyclophosphamide demonstrated a trend toward improved event-free survival when compared with those treated with corticosteroids and azathioprine; in particular, at 10 years, 56% of patients treated with cyclophosphamide were event-free compared with 15% of those treated with azathioprine.


This large case series has added to our understanding of the clinical manifestations of neuro-BD and suggests that the presence of HLA B51 is associated with worse long-term outcomes. It is also interesting that patients who received corticosteroids and intravenous cyclophosphamide had a trend toward lower rates of flares. A caveat is that the retrospective, case-series nature of this study limits the robustness of these findings; however, owing to the relative rarity of the disease, randomized controlled trials will be exceedingly difficult to conduct, and therefore such case series may be the best information available to guide therapy. An area for future research suggested by these findings is how HLA B51 testing can be used in the management of patients with neuro-BD. For example, should all patients with neuro-BD undergo HLA B51 testing to inform their prognosis? Also, because of some success with the anti-tumor necrosis factor agents in treating the ocular manifestations of BD,[3] it would be of interest to know the potential effect of these agents on neuro-BD.



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