Clinical characteristics of neuro-Behcet's disease in Japan: a multicenter retrospective analysis

Abstract

To delineate the clinical characteristics of neuro-Behçet's disease (NBD), a multicenter retrospective survey was performed in BD patients who had presented any neurological manifestations between 1988 and 2008. The diagnosis of acute NBD, chronic progressive (CP) NBD, and non-NBD was confirmed by retrospective review of clinical records. Data on a total of 144 patients were collected; 76 with acute NBD, 35 with CP NBD, and 33 with non-NBD. High-intensity lesions on T2-weighted magnetic resonance imaging (MRI) were found in 60.5% of the patients with acute NBD, 54.2% with CP NBD, and 42.4% with non-NBD, whereas brainstem atrophy was observed in 7.5% with acute NBD, 71.4% with CP NBD, and 9.0% with non-NBD. The cerebrospinal fluid (CSF) cell count was prominently elevated in patients with acute NBD, but was normal in about 15% of those with CP NBD. The sensitivity and specificity of the CSF cell count for the diagnosis of acute NBD versus non-NBD were 97.4 and 97.0%, respectively (cut-off 6.2/mm(3)). The sensitivity and specificity of CSF interleukin (IL)-6 for the diagnosis of CP NBD versus the recovery phase of acute NBD were 86.7 and 94.7%, respectively (cut-off 16.55 pg/ml). The results indicate that elevation of the CSF cell count and CSF IL-6 and the presence of brainstem atrophy on MRI are useful for the diagnosis of NBD.

Fig. 1

Cerebrospinal fluid (CSF) in neuro-Behçet’s disease (NBD). CSF cell count (a), total protein (b), and glucose (c), in patients with acute NBD, chronic progressive (CP) NBD, and non-NBD. Statistical significance was analyzed by the Kruskal-Wallis test with multiple comparison

Fig. 2

Receiver operating characteristic (ROC) analysis of the CSF cell count and CSF interleukin-6 (IL-6) for the differential diagnosis of NBD. a, b ROC analysis of the CSF cell count for the differential diagnosis of acute NBD and CP NBD from non-NBD. The sensitivity and specificity of CSF cell counts for the diagnosis of acute NBD versus non-NBD were 97.4 and 97.0%, respectively, at the cut-off value of 6.2/mm³ (a) [area under the curve (AUC): 0.9984 (95% confidence interval; CI, = 0.9951–1.002), P < 0.0001], whereas the sensitivity and specificity of the CSF cell count for the diagnosis of CP NBD versus non-NBD were 68.6 and 97.0%, respectively, at the cut-off value of 6.0/mm³ [AUC: 0.9126 (95% CI = 0.8473–0.9778), P < 0.0001] (b). c, d ROC analysis of CSF IL-6 for the diagnosis of CP NBD. The sensitivity and specificity of CSF IL-6 for the differential diagnosis of CP NBD (n = 25) versus non-NBD (n = 12) were 96.0 and 100%, respectively, at the cut-off value of 5.5 pg/ml [AUC: 0.9767 (95% CI = 0.9292–1.024), P < 0.0001] (c), whereas the sensitivity and specificity of CSF IL-6 for the diagnosis of CP NBD (n = 25) versus acute NBD in the recovery phase (n = 19) were 92.0 and 94.7%, respectively, at the cut-off value of 16.55 pg/ml [AUC: 0.9411 (95% CI = 0.8626–1.020), P < 0.0001] (d)