Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort

Abstract

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.

Early predictors of new-onset immune-related seizures: a preliminary study

“…Brain MRI is one of the etiological examinations for initial epilepsy or encephalitis, especially the abnormal signal of T2/FLAIR in one or both medial temporal lobes could indicate AE [ 23 ]. Our study showed that 6/23 (26.1%) patients in the AE group had abnormal neuroimaging findings, supporting the findings of previous studies [ 24 ]. Although the results of multivariate analysis showed that brain MRI could not be used as an independent indicator for early prediction ( P = 0.807), the abnormal brain MRI lesions in a few patients with AE could be reversibly changed with immunotherapy (Fig.…”

A Rare Case of Anti-Ma2 Antibody-Mediated Autoimmune Encephalomyelitis in Childhood

“…However, reports of anti-Ma2-associated encephalitis in childhood are extremely rare, and an autoimmune origin appears to have been more likely in this case than a paraneoplastic mechanism [2]. To our knowledge, only seven pediatric cases of anti-Ma2-associated neurological symptoms have been reported to date (Table 1) [2][3][4][5]. There were three male and four female patients, with a median age of 4 years.…”

Anti-SOX1 Antibodies in a 3-Year-old Girl, Post-Varicella

“…The first involves a 17-year-old adolescent with paraneoplastic limbic encephalitis due to Hodgkin lymphoma, and the second concerns a 12-year-old-girl witch encephalitis, where oncological evaluation yielded negative results. 10,11 Here, we present a rare case of a 3-year-old girl with anti-SOX1 antibodies post-varicella, and no associated malignancy.…”