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Título

Clinical differences among children with seronegative NORSE and anti-NMDA encephalitis

Resumo

Introduction: NORSE patients often fulfill the criteria for possible AIE, nonetheless nearly half of the cases are cryptogenic. NORSE secondary to anti-NMDAR encephalitis have different treatment options and response rates, and data comparing both diseases are scarce. Objective: To compare clinical and paraclinical characteristics of pediatric patients with NORSE and anti-NMDAR. Methods: Chart of patients ≤ 13 years with NORSE that fulfill the AIE criteria included in BrAIN (Brazilian autoimmune encephalitis network) database from 2017 to 2022 were reviewed. Information on clinical, epidemiological and treatment data were compiled. NORSE was defined as new-onset refractory status epilepticus in patients without active epilepsy or other preexisting relevant neurological disorder, according to 2018 consensus. Seronegative NORSE were compared to a cohort of pediatric anti-NMDAR, using T-test and Chi square tests. Statistical significance was set at p<0,05. Results: We found 19 patients with NORSE, and 5 of them that had AIE antibodies identified were excluded. The remaining 14 patients constituted the pediatric NORSE cohort. There were no differences between groups regarding female gender (NORSE: 36% x NMDA: 56%, p=0.23) or age (NORSE: 6.64 ± 2.87 x NMDA:5.62 ± 3.27, p=0.3). Prodromal symptoms were more frequent in NORSE (NORSE: 93% x NMDA: 52%, p=0.009), of which the most common was fever (NORSE: 71% x NMDA: 37%, p=0.04). NORSE patients presented less frequently with behavioral, psychiatric, and cognitive changes (NORSE: 50% x NMDA: 96%, p=0.0005) and movement disorders (NORSE: 64% x NMDA: 93%, p=0.02), like orofacial dyskinesia (NORSE: 29% x NMDA: 44%, p=0.035), dystonia (NORSE: 0% x NMDA: 56%, p=0.0005) and chorea (NORSE: 0% x NMDA: 37%, p=0.0009). NORSE patients also showed less frequent CSF pleocytosis, defined as >5cels/mm³ (NORSE: 21% x NMDA: 52%, p = 0.059), and normal brain MRI (NORSE: 21% x NMDA: 64%, p = 0.009). Conclusions: We found that NORSE patients have more frequently prodromal symptoms. It’s possible that the high frequency of orofacial dyskinesia in NORSE patients might be result of misinterpretation of focal seizures. Moreover, movement disorders, behavioral and cognitive changes were more common in anti-NMDAR, indicating that cognitive symptoms may be a clue in differentiating anti-NMDAR from seronegative NORSE.

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Área

Neuroimunologia