Abstract
Neuro-Behçet's disease (NBD) is a serious complication of Behçet's disease. Generally, NBD patients with a chronic course are refractory to immunosuppressive treatment, resulting in the deterioration of personality. In this study, levels of B cell-activating factor belonging to the TNF family (BAFF) were measured in the cerebrospinal fluid (CSF) from 18 patients with NBD, 27 patients with epidemic aseptic meningitis (AM), 24 patients with multiple sclerosis (MS) and 34 healthy controls. BAFF levels in patients with NBD were significantly elevated compared with healthy controls, but showed no statistically significant elevation compared with either of the disease controls. In contrast, CSF IL-6 levels were slightly elevated in patients with NBD and significantly elevated in patients with AM and MS compared with healthy controls. Patients with NBD were subdivided into two groups according to their clinical course (eight patients with a slowly progressive course presenting with psychosis and dementia and 10 patients with an acute course including aseptic meningitis, brainstem involvement and myelopathy). BAFF levels were significantly increased in those with a slowly progressive course compared with those with an acute course. CSF BAFF levels did not correlate with serum BAFF levels, CSF cell counts or CSF IL-6 levels in patients with NBD. These data suggested that BAFF was produced within the central nervous system and may be associated with the development of NBD, particularly with a progressive course.
Highlights
Behcet’s disease (BD) is a systemic disease characterized primarily by recurrent oral and genital aphthous ulcers, uveitis and skin findings
cerebrospinal fluid (CSF) IL-6 levels were slightly elevated in patients with neuro-Behcet’s disease (NBD) and significantly elevated in patients with aseptic meningitis (AM) and multiple sclerosis (MS) compared with healthy controls
The results from this study showed that BAFF levels in CSF were elevated in patients with NBD, and were independent of serum BAFF levels
Summary
Behcet’s disease (BD) is a systemic disease characterized primarily by recurrent oral and genital aphthous ulcers, uveitis and skin findings. Involvement of the central nervous system (CNS), referred to as neuro-Behcet’s disease (NBD), is less frequent than the other major presentations, but is one of the most serious complications of BD. NBD can occur either as a result of inflammation within the CNS or of vascular complications. NBD shows a variety of manifestations, including aseptic meningitis, increased intracranial pressure, meningomyelitis, brainstem involvement, pyramidal signs, cerebellar signs, sensory signs, and psychosis or dementia [1,2,3]. The most serious symptom is slowly progressive dementia or psychosis, which is resistant to immunosuppressive treatments and can result in severe deterioration of the patients’ personality. Few immunological markers are known to be associated with the activity or pathogenesis of NBD [4, 5]
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