Hemiparesis was noticed in 2 cases, facial paralysis in 1 case, and opercular syndrome in 1 case
Hemiparesis was noticed in 2 cases, facial paralysis in 1 case, and opercular syndrome in 1 case. Brain MRI showed increased transmission on ICEC0942 HCl fluid-attenuated inversion recovery (FLAIR) and T2-weighted images in 9 patients. 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) transmission of the temporal lobe in 5 patients. Eighteen patients showed improvement following first-line immunotherapy (high-dose corticosteroids, intravenous immunoglobulin). One individual with anti-GAD encephalitis died despite escalating immunotherapy. Conclusion Diagnosis of autoimmune encephalitis is usually challenging in children, because of misleading presentations. An early and accurate diagnosis is usually important to enable proper therapeutic interventions. 1. Introduction Autoimmune encephalitis (AE) represents one of the most common causes of noninfectious encephalitis. In the past 10 years, an increasing quantity of AE cases have been reported [1]. The clinical presentation of AE in child years is subacute with a varied constellation of symptoms [2C4]. Brain magnetic resonance imaging (MRI) may demonstrate abnormalities that provide clues for diagnosis [2, 5]. The identification of specific autoantibodies was a major advance achieved in neurology. Seronegative AE had been reported [4]. The outcome of AE in child years is generally good [2]. In Tunisia, there was no published series of pediatric AE. The aim of the present study was to investigate clinical features, biological and radiological aspects, management, and end result of Tunisian children with AE. 2. Patients and Methods We conducted a retrospective and descriptive study over 17 years (between 2004 and 2020) in the Department of Child and Adolescent Neurology at the National Institute Mongi Ben Hmida of Neurology (Tunis, Tunisia). Patients with acute or subacute neurological disorders were considered eligible for this study if they fulfilled the consensus diagnostic criteria for autoimmune encephalitis in adults [1] and revised based on the newly proposed diagnostic criteria in pediatric patients [6]. The exclusion criteria included patients with evidence of infectious encephalitis, for example, viral, bacterial, Mycobacterium tuberculosis, or fungal. Antibodies were detected using indirect immunofluorescence by commercialized slides with a mosaic of biochips (Euroimmun?), each one made up ICEC0942 HCl of transfected cells expressing the receptors of a different neuronal surface antigen: NMDA, AMPA, GABAB, CASPR2, and LGI1. Antibodies against Cv2, Ma2, Ri, Yo, Hu, recoverin, titin, SOX1, and amphiphysin were tested by the commercial immunoblot kit EUROLINE Paraneoplastic Neurological Syndromes 12 Ag (DL 1111-1601-4 G; Euroimmun, Lbeck, Germany) following the manufacturers’ instructions at serum dilution 1/100. Antibodies against GAD65 were detected using a commercialized enzyme-linked immunosorbent assay from Euroimmun?. Medical records of patients with AE were retrospectively examined. Demographic characteristics, clinical data, biological findings, characteristics of brain magnetic resonance imaging (MRI), and the data about therapeutic management and end result were collected. First-line immunotherapy included intravenous (IV) methylprednisolone or intravenous immunoglobulins (IVIG), or a combination of these. Rituximab or azathioprine was defined as second-line immunotherapy. All patients were followed for at least 3 months (in the range of 3 months-9.5 years). Good end result was defined as no sequela, and poor end result as having any sequela. A descriptive analysis was performed using SPSS software. Data are expressed as means. 3. Results Nineteen children were included in our study. The male-female ratio was 0.58 (12 ladies and 7 males). Based on the proposed diagnostic criteria for ICEC0942 HCl autoimmune encephalitis [1, 6], all of the patients ICEC0942 HCl ICEC0942 HCl met a definite diagnosis of autoimmune encephalitis. Antibodies were detected against NMDAR in 11 cases, against Ma2 in 4 cases, against GAD65 in 3 cases, and against SOX1 in Kcnj12 one case. The median age at diagnosis was 7.68 years (range: 10 months-13 years). There was a personnel medical history of neurofibromatosis type 1 (NF1) in one case with anti-NMDAR encephalitis, epileptic encephalopathy in 1 case with anti-Ma2 encephalitis, and febrile seizure in one case with anti-GAD65 encephalitis. The majority of patients experienced subacute onset of symptoms. Eight patients presented with prodromal symptoms, including fever and headache. Two cases with anti-NMDAR encephalitis experienced previous herpes simplex encephalitis diagnosed by polymerase chain reaction (PCR). The most preponderant clinical manifestations were seizures, observed in 18 cases, and behavioral disturbances, noticed in all cases. Seizures were focal in 11 cases and generalized in 7 cases. On examination, a decreased level of consciousness was observed in 11 cases and cognitive dysfunction in 12 cases. Speech disturbances were noted in 12 cases. Movement disorders were objectified in 8 cases with anti-NMADR encephalitis, 1 case with anti-GAD65 encephalitis, and 1 case with anti-SOX1 encephalitis. There were 8 cases of orofacial dyskinesia, 4 cases of dystonia, 2 cases of tremor, 1 case of myoclonia, and 1 case.