Limbic Encephalitis in Taiwanese Children and Adolescence: A Single Center Study
Non-viral limbic encephalitis, which may be paraneoplastic or idiopathic, is increasingly recognized in adults and children. Early identification of potential patients, who have neuronal autoantibodies to intracellular or neuronal surface antigens in order to give appropriate immunotherapy, is key t...
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| Format: | Article |
| Language: | English |
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Elsevier
2013-08-01
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| Series: | Pediatrics and Neonatology |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S187595721300017X |
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| author | I-Jun Chou Huei-Shyong Wang Jainn-Jim Lin Chang-Fu Kuo Kuang-Lin Lin Min-Liang Chou Po-Cheng Hung Meng-Ying Hsieh Yun-Tong Lin |
| author_facet | I-Jun Chou Huei-Shyong Wang Jainn-Jim Lin Chang-Fu Kuo Kuang-Lin Lin Min-Liang Chou Po-Cheng Hung Meng-Ying Hsieh Yun-Tong Lin |
| author_sort | I-Jun Chou |
| collection | DOAJ |
| description | Non-viral limbic encephalitis, which may be paraneoplastic or idiopathic, is increasingly recognized in adults and children. Early identification of potential patients, who have neuronal autoantibodies to intracellular or neuronal surface antigens in order to give appropriate immunotherapy, is key to improving the prognosis. This cross-sectional study describes the clinical manifestation and the serological evidence of the presence of neuronal antibodies in Taiwanese children with limbic encephalitis.
Method: We enrolled children and adolescents who had been hospitalized due to nonviral limbic encephalitis. Serum samples from these patients were collected to screen antibodies against intracellular antigens [amphiphysin, Ma2, Ri, Yo, Hu and antiglutamic acid decarboxylase (GAD)] and neuronal surface antigens [N-methyl-d-aspartate (NMDA) receptor, γ-amino butyric acid (GABAB) receptor and voltage-gated potassium channel complexes (VGKCs)].
Results: All of the 10 enrolled patients had acute onset of fever and rapid clinical deterioration. They had persistent neuropsychiatric symptoms and 90% developed refractory epilepsy, despite six patients having been treated with methylprednisolone pulse therapy or intravenous immunoglobulin (IVIG) at the acute stage. In the laboratory findings, half of the cases were positive for antibodies with regards to intracellular antigens (amphiphysin or GAD). The general outcomes, assessed by Glasgow Outcome Scale, were similar between patients with and those without the antibodies (Mann-Whitney U test, p = 0.43). One patient, who was positive for antibodies to amphiphysin 10 years after disease onset, still had a significant response to oral prednisolone therapy. At the end of the follow-up period, no cancer or insulin-dependent diabetes mellitus was detected in any of the patients.
Conclusion: This study provides evidence for a potential association between antibodies and limbic encephalitis. The presence of antibodies, especially antibodies to GAD, may serve as an indicator for immunotherapy. |
| format | Article |
| id | doaj-art-2bf3e078abab4f1a837b62ddd06de411 |
| institution | OA Journals |
| issn | 1875-9572 |
| language | English |
| publishDate | 2013-08-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Pediatrics and Neonatology |
| spelling | doaj-art-2bf3e078abab4f1a837b62ddd06de4112025-08-20T02:17:04ZengElsevierPediatrics and Neonatology1875-95722013-08-0154424625310.1016/j.pedneo.2013.01.016Limbic Encephalitis in Taiwanese Children and Adolescence: A Single Center StudyI-Jun Chou0Huei-Shyong Wang1Jainn-Jim Lin2Chang-Fu Kuo3Kuang-Lin Lin4Min-Liang Chou5Po-Cheng Hung6Meng-Ying Hsieh7Yun-Tong Lin8Division of Pediatric Neurology, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taoyuan, TaiwanDivision of Pediatric Neurology, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taoyuan, TaiwanDivision of Pediatric Neurology, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taoyuan, TaiwanDivision of Rheumatology, Allergy, and Immunology, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taoyuan, TaiwanDivision of Pediatric Neurology, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taoyuan, TaiwanDivision of Pediatric Neurology, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taoyuan, TaiwanDivision of Pediatric Neurology, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taoyuan, TaiwanDivision of Pediatric Neurology, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taoyuan, TaiwanDivision of Pediatric Neurology, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taoyuan, TaiwanNon-viral limbic encephalitis, which may be paraneoplastic or idiopathic, is increasingly recognized in adults and children. Early identification of potential patients, who have neuronal autoantibodies to intracellular or neuronal surface antigens in order to give appropriate immunotherapy, is key to improving the prognosis. This cross-sectional study describes the clinical manifestation and the serological evidence of the presence of neuronal antibodies in Taiwanese children with limbic encephalitis. Method: We enrolled children and adolescents who had been hospitalized due to nonviral limbic encephalitis. Serum samples from these patients were collected to screen antibodies against intracellular antigens [amphiphysin, Ma2, Ri, Yo, Hu and antiglutamic acid decarboxylase (GAD)] and neuronal surface antigens [N-methyl-d-aspartate (NMDA) receptor, γ-amino butyric acid (GABAB) receptor and voltage-gated potassium channel complexes (VGKCs)]. Results: All of the 10 enrolled patients had acute onset of fever and rapid clinical deterioration. They had persistent neuropsychiatric symptoms and 90% developed refractory epilepsy, despite six patients having been treated with methylprednisolone pulse therapy or intravenous immunoglobulin (IVIG) at the acute stage. In the laboratory findings, half of the cases were positive for antibodies with regards to intracellular antigens (amphiphysin or GAD). The general outcomes, assessed by Glasgow Outcome Scale, were similar between patients with and those without the antibodies (Mann-Whitney U test, p = 0.43). One patient, who was positive for antibodies to amphiphysin 10 years after disease onset, still had a significant response to oral prednisolone therapy. At the end of the follow-up period, no cancer or insulin-dependent diabetes mellitus was detected in any of the patients. Conclusion: This study provides evidence for a potential association between antibodies and limbic encephalitis. The presence of antibodies, especially antibodies to GAD, may serve as an indicator for immunotherapy.http://www.sciencedirect.com/science/article/pii/S187595721300017XamphiphysinGADlimbic encephalitisseizure |
| spellingShingle | I-Jun Chou Huei-Shyong Wang Jainn-Jim Lin Chang-Fu Kuo Kuang-Lin Lin Min-Liang Chou Po-Cheng Hung Meng-Ying Hsieh Yun-Tong Lin Limbic Encephalitis in Taiwanese Children and Adolescence: A Single Center Study Pediatrics and Neonatology amphiphysin GAD limbic encephalitis seizure |
| title | Limbic Encephalitis in Taiwanese Children and Adolescence: A Single Center Study |
| title_full | Limbic Encephalitis in Taiwanese Children and Adolescence: A Single Center Study |
| title_fullStr | Limbic Encephalitis in Taiwanese Children and Adolescence: A Single Center Study |
| title_full_unstemmed | Limbic Encephalitis in Taiwanese Children and Adolescence: A Single Center Study |
| title_short | Limbic Encephalitis in Taiwanese Children and Adolescence: A Single Center Study |
| title_sort | limbic encephalitis in taiwanese children and adolescence a single center study |
| topic | amphiphysin GAD limbic encephalitis seizure |
| url | http://www.sciencedirect.com/science/article/pii/S187595721300017X |
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