Makimoto Go, Manabe Yasuhiro, Yamakawa Chizuru, Fujii Daiki, Ikeda-Sakai Yasuko, Narai Hisashi, Omori Nobuhiko, Abe Koji
Department of Neurology, National Hospital Organization Okayama Medical Center;
Neurol Int. 2012 Jan 9;4(1):e5. doi: 10.4081/ni.2012.e5. Epub 2012 Feb 9.
We report 2 cases that were considered to be neuro-Sweet disease. They initially manifested with meningoencephalitis and no skin lesions, and rapidly improved with corticosteroid therapy. In both cases, patients complained of meningitic symptoms such as fever and headache, and HLA-B54 and -Cw1 turned out to be positive over the clinical course. Cerebrospinal fluid analysis showed increased levels of lymphocytes and protein. In case #1, fluid-attenuated inversion recovery (FLAIR), magnetic resonance imaging (MRI) and diffusion-weighted images (DWI) showed high-intensity signals in the right dorsal medulla oblongata, bilateral dorsal midbrain, and left thalamus. In case #2, FLAIR and DWI showed high-intensity signals in the bilateral cerebellar cortex and left caudate nucleus. Symptoms and MRI images were markedly improved in both cases after corticosteroid pulse therapy. According to published diagnostic criteria, these 2 cases were considered possible neuro-Sweet disease. These cases suggest that the combination of meningoencephalitis and HLA specificity is important to consider the possibility of neuro-Sweet disease, even without skin lesions.
我们报告了2例被认为是神经白塞病的病例。它们最初表现为脑膜脑炎且无皮肤损害，经皮质类固醇治疗后迅速好转。在这两个病例中，患者均主诉有发热和头痛等脑膜症状，且在临床病程中HLA - B54和 - Cw1结果呈阳性。脑脊液分析显示淋巴细胞和蛋白质水平升高。在病例1中，液体衰减反转恢复序列（FLAIR）、磁共振成像（MRI）和扩散加权成像（DWI）显示右侧延髓背侧、双侧中脑背侧和左侧丘脑有高强度信号。在病例2中，FLAIR和DWI显示双侧小脑皮质和左侧尾状核有高强度信号。皮质类固醇脉冲治疗后，两个病例的症状和MRI图像均明显改善。根据已发表的诊断标准，这2例被认为可能是神经白塞病。这些病例表明，即使没有皮肤损害，脑膜脑炎与HLA特异性的结合对于考虑神经白塞病的可能性也很重要。
