系统性红斑狼疮1例,表现为小脑可逆性水肿病变。

Ryumachi. [Rheumatism] Pub Date : 2002-10-01
Michio Hanyuda, Yoshiyuki Yoda, Takayuki Shiozawa, Ryosuke Hanaoka, Yusuke Miwa, Shuji Kaga, Takeshi Kasama, Masao Negishi, Hirotsugu Ide
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引用次数: 0

摘要

我们报告一例24岁的女性与系统性红斑狼疮(SLE)。患者于1996年12月出现颈部红斑及多发性关节痛。根据高水平的抗核抗体和抗双链dna抗体阳性,我们诊断患者患有SLE。她的症状得到改善,在类固醇治疗后病情得以维持。2000年8月,患者突然出现头痛、恶心、眩晕、小脑性共济失调、固定性眼球震颤、意向性震颤。她抗磷脂抗体呈阴性。脑脊液IgG指数和IL-6水平均较高。右侧小脑半球MRI显示t1增强图像为等信号强区,t2增强图像为弥漫性边界不清的高信号强区。脑单光子发射计算机断层扫描(SPECT)观察到磁共振成像(MRI)检测到的小脑病变相应部位脑血流量增加。中枢神经系统(CNS)狼疮被证实在小脑病变的存在。经类固醇脉冲治疗后,脑MRI和SPECT异常立即消失,步态紊乱等症状得到改善。该患者被诊断为急性神经精神性SLE,伴有小脑症状,这种症状很少被观察到为SLE的局部神经征象。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渗透性的增加。
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[A case with systemic lupus erythematosus presenting with reversible edematous lesion in cerebellum].

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.

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