UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

Centromedian thalamic nuclei deep brain stimulation and Anakinra treatment for FIRES - Two different outcomes

Sa, M; Singh, R; Pujar, S; D'Arco, F; Desai, N; Eltze, C; Hughes, E; ... Valentin, A; + view all (2019) Centromedian thalamic nuclei deep brain stimulation and Anakinra treatment for FIRES - Two different outcomes. European Journal of Paediatric Neurology , 23 (5) pp. 749-754. 10.1016/j.ejpn.2019.08.001. Green open access

[img]
Preview
Text
Sa et al. CMN-DBS and Anakinra treatment for FIRES - highlighted.pdf - Accepted version

Download (626kB) | Preview

Abstract

Febrile infection-related epilepsy syndrome (FIRES) is a severe epilepsy disorder that affects previously healthy children. It carries high likelihood of unfavourable outcome and putative aetiology relates to an auto-inflammatory process. Standard antiepileptic drug therapies including intravenous anaesthetic agents are largely ineffective in controlling status epilepticus in FIRES. Deep brain stimulation of the centromedian thalamic nuclei (CMN-DBS) has been previously used in refractory status epilepticus in only a few cases. The use of Anakinra (a recombinant version of the human interleukin-1 receptor antagonist) has been reported in one case with FIRES with good outcome. Here we describe two male paediatric patients with FIRES unresponsive to multiple anti-epileptic drugs, first-line immune modulation, ketogenic diet and cannabidiol. They both received Anakinra and underwent CMN-DBS. The primary aim for CMN-DBS therapy was to reduce generalized seizures. CMN-DBS abolished generalized seizures in both cases and Anakinra had a positive effect in one. This patient had a favourable outcome whereas the other did not. These are the first reported cases of FIRES where CMN-DBS has been used.

Type: Article
Title: Centromedian thalamic nuclei deep brain stimulation and Anakinra treatment for FIRES - Two different outcomes
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.ejpn.2019.08.001
Publisher version: https://doi.org/10.1016/j.ejpn.2019.08.001
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: FIRES; deep brain stimulation; centromedian thalamic nuclei; anakinra
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Neurosciences Dept
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10086983
Downloads since deposit
59Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item