Huss G P, Mattle H, Wessel K, Mumenthaler M
Neurologische Klinik, Inselspital, Universität Bern.
Nervenarzt. 1992 Jun;63(6):352-8.
The value of brain CT-scan, magnetic resonance imaging (MRI) and angiography for diagnosis, differential diagnosis and follow up in Neuro-Behçet-Syndrome is assessed in 5 cases. Three of the patients presented with clinical signs of encephalitis. Further investigations led to the diagnosis of Behçet-Syndrome. CT-scan was negative in two of these cases, but MRI showed multiple, predominantly periventricular lesions with high signal intensity on T2-weighted spin-echo images in all three. Clinical symptoms improved with steroid and chlorambucil therapy in all three cases. In two patients the MRI-lesions resolved at least partially after 1.5 and 3 years of treatment respectively. In one patient the initial MRI-findings were still present after 7 months of treatment. The other two patients presented with headache, papilledema and increased CSF-pressure. The cause was superior sagittal sinus thrombosis, confirmed by angiography in both cases. Additional symptoms appeared later and led to the diagnosis of Behçet syndrome. One patient died of pulmonary aneurysms 28 months after the diagnosis had been established. The course of disease of the remaining patient is so far favorable.
评估了5例神经白塞综合征患者脑部CT扫描、磁共振成像（MRI）和血管造影在诊断、鉴别诊断及随访中的价值。其中3例患者出现脑炎的临床症状。进一步检查后确诊为白塞综合征。这3例中2例CT扫描结果为阴性，但MRI显示所有3例在T2加权自旋回波图像上均有多个主要位于脑室周围的高信号病变。所有3例患者经类固醇和苯丁酸氮芥治疗后临床症状均有改善。2例患者分别在治疗1.5年和3年后MRI病变至少部分消退。1例患者在治疗7个月后最初的MRI表现仍存在。另外2例患者出现头痛、视乳头水肿和脑脊液压力升高。病因是上矢状窦血栓形成，2例均经血管造影证实。随后出现其他症状并确诊为白塞综合征。1例患者在确诊后28个月死于肺动脉瘤。目前，其余患者的病程良好。
