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

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Mario Sa, Rinki Singh, Suresh Pujar, Felice D'arco, Nivedita Desai, Christin Eltze, Elaine Hughes, Muthana Al Obaidi, Despina Eleftheriou, Martin Tisdall, Richard Selway, J. Helen Cross, Marios Kaliakatsos, Antonio Valentin

Original languageEnglish
Pages (from-to)749-754
JournalEuropean Journal of Paediatric Neurology
Volume23
Issue number5
Early online date8 Aug 2019
DOIs
Accepted/In press1 Aug 2019
E-pub ahead of print8 Aug 2019
Published1 Sep 2019

King's Authors

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.

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