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CC BY-NC-ND 4.0 · Neuropediatrics 2024; 55(01): 009-015
DOI: 10.1055/s-0043-1774318
Original Article

Spectrum, Evolution, and Clinical Relationship of Magnetic Resonance Imaging in 31 Children with Febrile Infection-Related Epilepsy Syndrome

Darinka Moreno-Brauer
1   Department of Neuropediatrics, University Medical Center Schleswig-Holstein, Kiel University (CAU), Kiel, Germany
,
Martin Häusler 
2   Division of Neuropediatrics and Social Pediatrics, Department of Pediatrics, University Hospital, RWTH Aachen, Aachen, Germany
,
Gerhard Kluger
3   Clinic for Neuropediatrics and Neurorehabilitation, Epilepsy Center for Children and Adolescents, Schön Clinic Vogtareuth, Germany
4   Research Institute for Rehabilitation, Transition, and Palliation, Paracelsus Medical University, Salzburg, Austria
,
Johannes Hensler   *
5   Department of Radiology and Neuroradiology, University Medical Center Schleswig-Holstein, Kiel University (CAU), Kiel, Germany
,
Andreas van Baalen    *
1   Department of Neuropediatrics, University Medical Center Schleswig-Holstein, Kiel University (CAU), Kiel, Germany
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Abstract

Objective Describing spectrum, evolution, and clinical relationship of brain magnetic resonance imaging (MRI) findings in a large case series of children with febrile infection-related epilepsy syndrome (FIRES).

Methods This retrospective study included 31 children with FIRES. Clinical data and MRI findings of the brain were evaluated. Poor clinical outcome was defined as severe disability, persistent vegetative state or stupor, very low intelligence quotient (<80), or death (modified Rankin scale 4–6 and Glasgow Outcome Score 1–3).

Results Seventeen (54.8%) children with FIRES showed no abnormalities in the initial MRI, whereas 28 (90.3%) children showed MRI abnormalities at follow-up. The most frequent abnormalities were brain atrophy (74.2%) and T2/fluid-attenuated inversion recovery changes (64.5%), mostly hippocampal (45.2%). Generalized brain atrophy was the most frequent type of atrophy (58%). The earliest atrophy was recorded 9 days after the onset of disease. It progressed even beyond the acute phase in most children (51.6%). The exploratory data analysis revealed nominal significance between all MRI abnormalities considered together and poor outcome (p = 0.049) and between generalized brain atrophy and anesthesia (p = 0.024). After adjustment for multiple testing, the p-values were not significant. The outcome in four (12.9%) children was not poor despite generalized brain atrophy.

Conclusion In contrast to the uniform clinical course, MRI demonstrated a broad spectrum of findings. Initially, these were mostly normal and therefore indicative of FIRES but then changed rapidly and were mostly progressive despite the stable chronic course. The cause may be ongoing disease, treatment intensity, or both. Future studies should focus on what process underlies the onset and the progression of brain atrophy. However, brain atrophy was not always related to poor outcomes in children despite FIRES.

Keywords

FIRES - status epilepticus - magnetic resonance imaging - anesthesia - brain atrophy - children

* These authors contributed equally to this work. Location where the work was performed: Department of Neuropediatrics, University Medical Center Schleswig-Holstein, Kiel University (CAU), Kiel, Germany


Supplementary Material



Publication History

Received: 18 May 2023

Accepted: 14 July 2023

Article published online:
05 October 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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