No evidence of neuronal/glial autoantibodies in febrile infection-related epilepsy syndrome (FIRES): a prospective clinic-serologic analysis

Frontiers in Neuroscience

Neuroscience

Gerhard Kluger¹  Frank Leypoldt²  Andreas van Baalen³  Janina Soler Wenglein³  Klaus-Peter Wandinger⁴ 

[1] Clinic for Neuropediatrics and Neurorehabilitation, Epilepsy Center for Children and Adolescents, Schön Clinic Vogtareuth, Vogtareuth, Germany;Research Institute for Rehabilitation, Transition, and Palliation, Paracelsus Medical University Salzburg, Salzburg, Austria;Department of Neurology, University Medical Center Schleswig-Holstein, Kiel University (CAU), Kiel, Germany;Neuroimmunology Section, Institute of Clinical Chemistry University Medical Center Schleswig-Holstein, Kiel, Germany;Department of Neuropediatrics, University Medical Center Schleswig-Holstein, Kiel University (CAU), Kiel, Germany;Neuroimmunology Section, Institute of Clinical Chemistry University Medical Center Schleswig-Holstein, Kiel, Germany;

关键词: FIRES;    NORSE;    status epilepticus;    encephalopathy;    autoimmune;    inflammation;    AE;    autoantibodies;   

DOI  :  10.3389/fnins.2023.1221761

 received in 2023-05-12, accepted in 2023-06-27,  发布年份 2023

来源: Frontiers

【 摘 要 】

The pediatric febrile infection-related epilepsy syndrome (FIRES) manifests with encephalopathy with super-refractory status epilepticus (SE) a few days after or accompanying a febrile illness. It often results in refractory epilepsy and cognitive dysfunction in previously healthy children and adolescents. The underlying pathomechanism is unknown, which is why causative neuronal and/or synaptic antibodies have been discussed. We report a prospective consecutive cohort of 14 children (10 male, four female) diagnosed with FIRES in the acute phase, whose serum and CSF were comprehensively screened for underlying synaptic/neuronal autoantibodies. The median age at onset was 6  years (range 4–9  years). None of the children had a medical history of epilepsy. Duration of SE varied from less than 1 week to 2.5  months (Median: 1  month, range  < 1  week-2.5  months). Clinical response to treatment with antiseizure medications was poor as well as the outcome: one child died in the acute phase of SE, and two died in the long term. All surviving children showed neuropsychological impairments. No underlying synaptic or neuronal autoantibodies were identified in 13 of 14 children’s sera or CSF. One child had currently uncharacterized neuronal autoantibodies in CSF, yet clinical presentation was atypical for FIRES. Based on our findings, the child was later diagnosed with autoimmune encephalitis (AE). We conclude that FIRES is not an autoantibody-mediated disease. However, a comprehensive screening for known and yet unknown antineuronal antibodies in serum and CSF is warranted to rule out AE mimicking FIRES.

【 授权许可】

Unknown   
Copyright © 2023 Soler Wenglein, Kluger, Leypoldt, Wandinger and van Baalen.