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Eur J Paediatr Neurol. 2019 Sep;23(5):749-754. doi: 10.1016/j.ejpn.2019.08.001. Epub 2019 Aug 8.

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

Author information

1
Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK.
2
Department of Clinical Neurophysiology, King's College Hospital NHS Trust, London, UK.
3
Department of Neuroradiology, Great Ormond Street Hospital for Children, London, UK.
4
Department of Paediatric Neurology, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
5
Department of Paediatric Rheumatology, Great Ormond Street Hospital for Children, London, UK.
6
Infection, Immunity and Rheumatology Section, University College London Great Ormond Street Institute of Child Health, London, UK.
7
Department of Neurosurgery, Great Ormond Street Hospital for Children, London, UK.
8
Department of Neurosurgery, King's College Hospital NHS Trust, London, UK.
9
Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK; Clinical Neurosciences, University College London NIHR BRC Great Ormond Street Institute of Child Health, London, UK.
10
Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK. Electronic address: marios.kaliakatsos@gosh.nhs.uk.

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.

KEYWORDS:

Anakinra; Centromedian thalamic nuclei; Deep brain stimulation; FIRES

PMID:
31446001
DOI:
10.1016/j.ejpn.2019.08.001
[Indexed for MEDLINE]

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