Hanyuda Michio, Yoda Yoshiyuki, Shiozawa Takayuki, Hanaoka Ryosuke, Miwa Yusuke, Kaga Shuji, Kasama Takeshi, Negishi Masao, Ide Hirotsugu
Department of Internal Medicine, Division of Rheumatology and Clinical Immunology, Showa University, Tokyo.
Ryumachi. 2002 Oct;42(5):801-6.
We report the case of a 24-year-old woman with systemic lupus erythematosus (SLE). The patient presented with cervical erythema and multiple arthralgia in December, 1996. Based on the high level of antinuclear antibody and the positivity for anti-double-stranded-DNA antibody, we diagnosed the patient as having SLE. Her symptoms improved and her condition was maintained following steroid treatment. In August 2000, the patient suddenly had headache, nausea, vertigo, cerebellar ataxia, fixation nystagmus, and intention tremor. She was negative for the anti-phospholipid antibody. The cerebrospinal fluid IgG index and the IL-6 level were high. MRI of the right cerebellar hemisphere showed an equal-signal-intensity region in the T 1-enhanced image, and a high-signal-intensity region with a diffuse undefined border in the T 2-enhanced image. The increased cerebral blood flow at the site corresponding to a cerebellar lesion detected by magnetic resonance imaging (MRI) was observed by brain single photon emission computed tomography (SPECT). The central nervous system (CNS) lupus was confirmed by the presence of a lesion in the cerebellum. The abnormalities detected in MRI and SPECT images of the brain disappeared immediately after the steroid pulse therapy, and symptoms such as ataxic gait were improved. This patient was diagnosed as having acute neuropsychiatric SLE with cerebellar symptoms that are rarely observed as a localized neural sign of SLE. The MRI and SPECT images suggested the presence of an inflammatory edematous lesion that was confined in the cerebellar hemisphere. This is considered to be due to the increase of vasopermeability.
我们报告了一例24岁的系统性红斑狼疮(SLE)女性患者。该患者于1996年12月出现颈部红斑和多处关节痛。基于抗核抗体水平升高及抗双链DNA抗体阳性,我们诊断该患者患有SLE。经类固醇治疗后,她的症状改善且病情得以维持。2000年8月,该患者突然出现头痛、恶心、眩晕、小脑性共济失调、凝视性眼球震颤和意向性震颤。她的抗磷脂抗体检测为阴性。脑脊液IgG指数和IL - 6水平升高。右侧小脑半球的MRI显示,在T1增强图像中有等信号强度区域,在T2增强图像中有边界不清的高信号强度区域。通过脑单光子发射计算机断层扫描(SPECT)观察到,磁共振成像(MRI)检测到的小脑病变对应部位脑血流增加。小脑出现病变证实了中枢神经系统(CNS)狼疮。在类固醇脉冲治疗后,脑部MRI和SPECT图像中检测到的异常立即消失,共济失调步态等症状也得到改善。该患者被诊断为患有急性神经精神性SLE,伴有小脑症状,而小脑症状作为SLE的局部神经体征很少见。MRI和SPECT图像提示存在局限于小脑半球的炎性水肿病变。这被认为是由于血管通透性增加所致。