Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort
Background. Autoimmune encephalitis (AE) is a rapidly progressive encephalopathy caused by antibodies targeting neurons in the central nervous system generating specific immune responses. It is increasingly recognized in children. Objective. To describe clinical, neuroimaging, and laboratory feature...
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| Format: | Article |
| Language: | English |
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Wiley
2021-01-01
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| Series: | Journal of Immunology Research |
| Online Access: | http://dx.doi.org/10.1155/2021/6666117 |
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| author | Bissene Douma Thouraya Ben Younes Hanene Benrhouma Zouhour Miladi Imen Zamali Aida Rouissi Hedia Klaa Ichraf Kraoua Melika Ben Ahmed Ilhem Ben Youssef Turki |
| author_facet | Bissene Douma Thouraya Ben Younes Hanene Benrhouma Zouhour Miladi Imen Zamali Aida Rouissi Hedia Klaa Ichraf Kraoua Melika Ben Ahmed Ilhem Ben Youssef Turki |
| author_sort | Bissene Douma |
| collection | DOAJ |
| description | Background. Autoimmune encephalitis (AE) is a rapidly progressive encephalopathy caused by antibodies targeting neurons in the central nervous system generating specific immune responses. It is increasingly recognized in children. Objective. To describe clinical, neuroimaging, and laboratory features, treatment, and outcome in a cohort of Tunisian children with AE. Methods. We conducted a retrospective review of the medical records of all children attending the Department of Child and Adolescent Neurology (Tunis) with autoimmune encephalitis between 2004 and 2020. Clinical, neuroimaging, laboratory features, therapeutic data, and outcome were analyzed. Results. Nineteen children were included in the study (12 girls and 7 boys). The median age at diagnosis was 7.68 years (range: 10 months-13 years). The most frequent manifestations were seizures and behavioral disorders. Eleven cases were diagnosed with anti-NMDA receptor encephalitis, 4 cases with anti-Ma2 encephalitis, 3 cases with anti-GAD encephalitis, and 1 case with anti-SOX1 encephalitis. Brain MRI showed increased T2 and fluid-attenuated inversion recovery (FLAIR) signal of the temporal lobe in 5 patients. Eighteen patients showed improvement following first-line immunotherapy (high-dose corticosteroids, intravenous immunoglobulin). One patient with anti-GAD encephalitis died despite escalating immunotherapy. Conclusion. Diagnosis of autoimmune encephalitis is challenging in children, because of misleading presentations. An early and accurate diagnosis is important to enable proper therapeutic interventions. |
| format | Article |
| id | doaj-art-e41b602af7574fbea3fbf76cd741638d |
| institution | Kabale University |
| issn | 2314-8861 2314-7156 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of Immunology Research |
| spelling | doaj-art-e41b602af7574fbea3fbf76cd741638d2025-08-20T03:26:33ZengWileyJournal of Immunology Research2314-88612314-71562021-01-01202110.1155/2021/66661176666117Autoimmune Encephalitis in Tunisia: Report of a Pediatric CohortBissene Douma0Thouraya Ben Younes1Hanene Benrhouma2Zouhour Miladi3Imen Zamali4Aida Rouissi5Hedia Klaa6Ichraf Kraoua7Melika Ben Ahmed8Ilhem Ben Youssef Turki9Research Laboratory LR18SP04 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, TunisiaResearch Laboratory LR18SP04 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, TunisiaResearch Laboratory LR18SP04 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, TunisiaResearch Laboratory LR18SP04 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, TunisiaUniversité de Tunis El Manar, Faculté de Médecine de Tunis, 1007 Tunis, TunisiaResearch Laboratory LR18SP04 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, TunisiaResearch Laboratory LR18SP04 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, TunisiaResearch Laboratory LR18SP04 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, TunisiaUniversité de Tunis El Manar, Faculté de Médecine de Tunis, 1007 Tunis, TunisiaResearch Laboratory LR18SP04 and Department of Child and Adolescent Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, TunisiaBackground. Autoimmune encephalitis (AE) is a rapidly progressive encephalopathy caused by antibodies targeting neurons in the central nervous system generating specific immune responses. It is increasingly recognized in children. Objective. To describe clinical, neuroimaging, and laboratory features, treatment, and outcome in a cohort of Tunisian children with AE. Methods. We conducted a retrospective review of the medical records of all children attending the Department of Child and Adolescent Neurology (Tunis) with autoimmune encephalitis between 2004 and 2020. Clinical, neuroimaging, laboratory features, therapeutic data, and outcome were analyzed. Results. Nineteen children were included in the study (12 girls and 7 boys). The median age at diagnosis was 7.68 years (range: 10 months-13 years). The most frequent manifestations were seizures and behavioral disorders. Eleven cases were diagnosed with anti-NMDA receptor encephalitis, 4 cases with anti-Ma2 encephalitis, 3 cases with anti-GAD encephalitis, and 1 case with anti-SOX1 encephalitis. Brain MRI showed increased T2 and fluid-attenuated inversion recovery (FLAIR) signal of the temporal lobe in 5 patients. Eighteen patients showed improvement following first-line immunotherapy (high-dose corticosteroids, intravenous immunoglobulin). One patient with anti-GAD encephalitis died despite escalating immunotherapy. Conclusion. Diagnosis of autoimmune encephalitis is challenging in children, because of misleading presentations. An early and accurate diagnosis is important to enable proper therapeutic interventions.http://dx.doi.org/10.1155/2021/6666117 |
| spellingShingle | Bissene Douma Thouraya Ben Younes Hanene Benrhouma Zouhour Miladi Imen Zamali Aida Rouissi Hedia Klaa Ichraf Kraoua Melika Ben Ahmed Ilhem Ben Youssef Turki Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort Journal of Immunology Research |
| title | Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort |
| title_full | Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort |
| title_fullStr | Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort |
| title_full_unstemmed | Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort |
| title_short | Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort |
| title_sort | autoimmune encephalitis in tunisia report of a pediatric cohort |
| url | http://dx.doi.org/10.1155/2021/6666117 |
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