Di Biasi C, Accorinti M, Trasimeni G, Pivetti Pezzi P, Melone A, Gualdi G
Istituto di Oftalmologia, Università La Sapienza, Roma.
Radiol Med. 1997 Apr;93(4):348-51.
Behçet's disease is a chronic relapsing disorder of unknown etiology characterized by oral aphthous ulcerations, uveitis, genital ulcerations and bone lesions. A variety of other signs including polyarthritis, vascular conditions (blood vessel occlusions and aneurysms), epididymitis, gastrointestinal, pulmonary and heart lesions may also occur. Central nervous system (CNS) involvement is reported in 10-49% of cases and it is the first symptom of the disease in 5% of subjects. The neuro-Behçet's syndrome may appear as a brainstem syndrome, meningoencephalitis and an organic confusional syndrome or dementia. Cranial hypertension, mostly related to cerebral venous thrombosis, is also present in neuro-Behçet's disease and its incidence is reported in up to 10% of Behçet's patients. MRI is reportedly the most sensitive neuroradiologic approach to detect the focal lesions related to neuro-Behçet's disease and several single cases or series of Behçet's patients with neurologic signs have been examined with MRI. We used MRI to investigate CNS involvement in Behçet's disease patients with and without previous neurologic signs. MRI was carried out on 17 patients with ocular Behçet's disease without neurologic symptoms to assess the possible subclinical involvement of the CNS. Cerebrospinal fluid spaces were enlarged in 8 patients and 5 patients exhibited cortical atrophy. PD and T2-weighted hypersignal foci were demonstrated in parietal, frontal, subcortical and periventricular white matter in 6 subjects. Neuroradiologic abnormalities were found only in the patients with complete disease and with the disease diagnosed more than 10 years earlier. Even though the pathogenesis of these neuroradiologic abnormalities and their correlation with Behçet's disease remain to be clarified, our study suggests the possibility of subclinical CNS involvement in these patients, which may affect the therapeutic approach and their prognosis.
白塞病是一种病因不明的慢性复发性疾病,其特征为口腔阿弗他溃疡、葡萄膜炎、生殖器溃疡和骨病变。还可能出现多种其他体征,包括多关节炎、血管病变(血管闭塞和动脉瘤)、附睾炎、胃肠道、肺部和心脏病变。据报道,10%-49%的病例会累及中枢神经系统(CNS),5%的患者以其作为疾病的首发症状。神经白塞综合征可能表现为脑干综合征、脑膜脑炎、器质性精神错乱综合征或痴呆。颅内高压主要与脑静脉血栓形成有关,在神经白塞病中也有出现,据报道在高达10%的白塞病患者中发生。据报道,MRI是检测与神经白塞病相关局灶性病变最敏感的神经放射学方法,已有数例或系列有神经体征的白塞病患者接受了MRI检查。我们使用MRI研究有无既往神经体征的白塞病患者的CNS受累情况。对17例无神经症状的眼部白塞病患者进行MRI检查,以评估CNS可能的亚临床受累情况。8例患者脑脊液间隙增宽,5例患者出现皮质萎缩。6例患者在顶叶、额叶、皮质下和脑室周围白质出现PD和T2加权高信号灶。仅在患有完全型疾病且诊断时间超过10年的患者中发现神经放射学异常。尽管这些神经放射学异常的发病机制及其与白塞病的相关性仍有待阐明,但我们的研究提示这些患者存在CNS亚临床受累的可能性,这可能会影响治疗方法及其预后。