Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation
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 turne...
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| Format: | Article |
| Language: | English |
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MDPI AG
2012-02-01
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| Series: | Neurology International |
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| Online Access: | http://www.pagepress.org/journals/index.php/ni/article/view/3175 |
| _version_ | 1797936266113712128 |
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| author | Go Makimoto Yasuhiro Manabe Chizuru Yamakawa Daiki Fujii Yasuko Ikeda-Sakai Hisashi Narai Nobuhiko Omori Koji Abe |
| author_facet | Go Makimoto Yasuhiro Manabe Chizuru Yamakawa Daiki Fujii Yasuko Ikeda-Sakai Hisashi Narai Nobuhiko Omori Koji Abe |
| author_sort | Go Makimoto |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-10T18:27:02Z |
| format | Article |
| id | doaj.art-c19d91279c31441793e7a3f32c1693f3 |
| institution | Directory Open Access Journal |
| issn | 2035-8385 2035-8377 |
| language | English |
| last_indexed | 2024-04-10T18:27:02Z |
| publishDate | 2012-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Neurology International |
| spelling | doaj.art-c19d91279c31441793e7a3f32c1693f32023-02-02T05:28:05ZengMDPI AGNeurology International2035-83852035-83772012-02-0141e5e510.4081/ni.2012.e51974Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestationGo Makimoto0Yasuhiro Manabe1Chizuru Yamakawa2Daiki Fujii3Yasuko Ikeda-Sakai4Hisashi Narai5Nobuhiko Omori6Koji Abe7Department of Neurology, National Hospital Organization Okayama Medical Center, OkayamaDepartment of Neurology, National Hospital Organization Okayama Medical Center, OkayamaDepartment of Neurology, National Hospital Organization Okayama Medical Center, OkayamaDepartment of Neurology, National Hospital Organization Okayama Medical Center, OkayamaDepartment of Neurology, National Hospital Organization Okayama Medical Center, OkayamaDepartment of Neurology, National Hospital Organization Okayama Medical Center, OkayamaDepartment of Neurology, National Hospital Organization Okayama Medical Center, OkayamaDepartment of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, OkayamaWe 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.http://www.pagepress.org/journals/index.php/ni/article/view/3175neuro-Sweet diseasemeningoencephalitisneuro-neutrophilic disease |
| spellingShingle | Go Makimoto Yasuhiro Manabe Chizuru Yamakawa Daiki Fujii Yasuko Ikeda-Sakai Hisashi Narai Nobuhiko Omori Koji Abe Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation Neurology International neuro-Sweet disease meningoencephalitis neuro-neutrophilic disease |
| title | Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation |
| title_full | Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation |
| title_fullStr | Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation |
| title_full_unstemmed | Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation |
| title_short | Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation |
| title_sort | two cases of possible neuro sweet disease with meningoencephalitis as the initial manifestation |
| topic | neuro-Sweet disease meningoencephalitis neuro-neutrophilic disease |
| url | http://www.pagepress.org/journals/index.php/ni/article/view/3175 |
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