Department of Rheumatology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374, Japan.
Mod Rheumatol. 2012 Jun;22(3):405-13. doi: 10.1007/s10165-011-0533-5. Epub 2011 Sep 21.
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.
为了描绘神经贝赫切特病（NBD）的临床特征，我们对 1988 年至 2008 年间出现任何神经系统表现的 BD 患者进行了一项多中心回顾性调查。急性 NBD、慢性进展性（CP）NBD 和非 NBD 的诊断通过回顾性临床记录进行确认。共收集了 144 例患者的数据；76 例为急性 NBD，35 例为 CP NBD，33 例为非 NBD。60.5%的急性 NBD、54.2%的 CP NBD 和 42.4%的非 NBD患者的 T2 加权磁共振成像（MRI）上有高强度病变，而急性 NBD 中有 7.5%、CP NBD 中有 71.4%、非 NBD 中有 9.0%的患者出现脑干萎缩。急性 NBD 患者的脑脊液（CSF）细胞计数明显升高，但 CP NBD 患者中约有 15%的细胞计数正常。CSF 细胞计数对急性 NBD 与非 NBD 的诊断的灵敏度和特异性分别为 97.4%和 97.0%（临界值为 6.2/mm(3)）。CSF 白细胞介素（IL）-6 对 CP NBD 与急性 NBD 恢复期的诊断的灵敏度和特异性分别为 86.7%和 94.7%（临界值为 16.55pg/ml）。结果表明，CSF 细胞计数和 CSF IL-6 的升高以及 MRI 上脑干萎缩对 NBD 的诊断具有一定的辅助作用。
