Clinical and cytokine profile of adult acute necrotizing encephalopathy
Background: Acute necrotizing encephalopathy (ANE), a fulminant encephalopathy, is often found in childhood. It is still uncertain whether adult patients with ANE display clinical features different from patients with typical pediatric onset. Furthermore, alterations in neuroinflammatory factors in...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2019-06-01
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| Series: | Biomedical Journal |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2319417017302032 |
| _version_ | 1818037595055063040 |
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| author | Yi-Ying Lin Kuang-Yung Lee Long-Sun Ro Yen-Shi Lo Chin-Chang Huang Kuo-Hsuan Chang |
| author_facet | Yi-Ying Lin Kuang-Yung Lee Long-Sun Ro Yen-Shi Lo Chin-Chang Huang Kuo-Hsuan Chang |
| author_sort | Yi-Ying Lin |
| collection | DOAJ |
| description | Background: Acute necrotizing encephalopathy (ANE), a fulminant encephalopathy, is often found in childhood. It is still uncertain whether adult patients with ANE display clinical features different from patients with typical pediatric onset. Furthermore, alterations in neuroinflammatory factors in patients with ANE have not been well-characterized. Here, we present an adult patient with ANE, and review all reported adult ANE cases in the literature. Methods: Serum levels of five cytokines were checked in an adult patient with ANE and compared with gender/age-matched controls. Literature search was performed with PubMed, using the term as “acute necrotizing encephalopathy” with the filter of adult 19 + years. Results: A total of 13 adult patients were reviewed. Compared with pediatric patients, adult ANE patients had similar clinical symptoms, biochemical data, and neuroimage findings, whereas adult ANE were more female-biased (female:male, 9:4) with a worse prognosis. Elevated cytokine levels in the serum and/or CSF is found in both adult-onset and pediatric-onset ANE. We found significantly elevated serum levels of IL-6 (17.17 pg/mL; healthy control: 1.43 ± 1.22 pg/mL) and VCAM-1 (3033.92 ng/mL; healthy control: 589.71 ± 133.13 ng/mL), and decreased serum TGF-β1 level (14.78 ng/mL, healthy controls: 25.81 ± 6.97 ng/mL) in our patient. Conclusions: Our findings clearly delineate the clinical features and further indicate the potential change in cytokine levels in adult patients with ANE, advancing our understanding of this rare disease. Keywords: Acute necrotizing encephalopathy, Adult, Cytokine, VCAM-1 |
| first_indexed | 2024-12-10T07:29:20Z |
| format | Article |
| id | doaj.art-31ada9b648c74288865fb63309fce843 |
| institution | Directory Open Access Journal |
| issn | 2319-4170 |
| language | English |
| last_indexed | 2024-12-10T07:29:20Z |
| publishDate | 2019-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Biomedical Journal |
| spelling | doaj.art-31ada9b648c74288865fb63309fce8432022-12-22T01:57:37ZengElsevierBiomedical Journal2319-41702019-06-01423178186Clinical and cytokine profile of adult acute necrotizing encephalopathyYi-Ying Lin0Kuang-Yung Lee1Long-Sun Ro2Yen-Shi Lo3Chin-Chang Huang4Kuo-Hsuan Chang5Department of Neurology, Chang Gung Memorial Hospital at Linkou, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, TaiwanDepartment of Neurology, Chang Gung Memorial Hospital at Keelung, Keelung, Taiwan; College of Medicine, Chang Gung University, Taoyuan, TaiwanDepartment of Neurology, Chang Gung Memorial Hospital at Linkou, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, TaiwanDepartment of Neurology, Chang Gung Memorial Hospital at Linkou, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, TaiwanDepartment of Neurology, Chang Gung Memorial Hospital at Linkou, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, TaiwanDepartment of Neurology, Chang Gung Memorial Hospital at Linkou, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan; Corresponding author. Department of Neurology, Chang Gung Memorial Hospital at Linkou, 5, Fusing St., Gueishan, Taoyuan 333, Taiwan.Background: Acute necrotizing encephalopathy (ANE), a fulminant encephalopathy, is often found in childhood. It is still uncertain whether adult patients with ANE display clinical features different from patients with typical pediatric onset. Furthermore, alterations in neuroinflammatory factors in patients with ANE have not been well-characterized. Here, we present an adult patient with ANE, and review all reported adult ANE cases in the literature. Methods: Serum levels of five cytokines were checked in an adult patient with ANE and compared with gender/age-matched controls. Literature search was performed with PubMed, using the term as “acute necrotizing encephalopathy” with the filter of adult 19 + years. Results: A total of 13 adult patients were reviewed. Compared with pediatric patients, adult ANE patients had similar clinical symptoms, biochemical data, and neuroimage findings, whereas adult ANE were more female-biased (female:male, 9:4) with a worse prognosis. Elevated cytokine levels in the serum and/or CSF is found in both adult-onset and pediatric-onset ANE. We found significantly elevated serum levels of IL-6 (17.17 pg/mL; healthy control: 1.43 ± 1.22 pg/mL) and VCAM-1 (3033.92 ng/mL; healthy control: 589.71 ± 133.13 ng/mL), and decreased serum TGF-β1 level (14.78 ng/mL, healthy controls: 25.81 ± 6.97 ng/mL) in our patient. Conclusions: Our findings clearly delineate the clinical features and further indicate the potential change in cytokine levels in adult patients with ANE, advancing our understanding of this rare disease. Keywords: Acute necrotizing encephalopathy, Adult, Cytokine, VCAM-1http://www.sciencedirect.com/science/article/pii/S2319417017302032 |
| spellingShingle | Yi-Ying Lin Kuang-Yung Lee Long-Sun Ro Yen-Shi Lo Chin-Chang Huang Kuo-Hsuan Chang Clinical and cytokine profile of adult acute necrotizing encephalopathy Biomedical Journal |
| title | Clinical and cytokine profile of adult acute necrotizing encephalopathy |
| title_full | Clinical and cytokine profile of adult acute necrotizing encephalopathy |
| title_fullStr | Clinical and cytokine profile of adult acute necrotizing encephalopathy |
| title_full_unstemmed | Clinical and cytokine profile of adult acute necrotizing encephalopathy |
| title_short | Clinical and cytokine profile of adult acute necrotizing encephalopathy |
| title_sort | clinical and cytokine profile of adult acute necrotizing encephalopathy |
| url | http://www.sciencedirect.com/science/article/pii/S2319417017302032 |
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