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

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

doi:10.1016/j.ejpn.2019.08.001

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

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

Authors

Affiliations

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: [email protected].

PMID: 31446001
DOI: 10.1016/j.ejpn.2019.08.001

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.

Publication types

Case Reports

MeSH terms

Acute Febrile Encephalopathy / complications
Acute Febrile Encephalopathy / therapy*
Anticonvulsants / therapeutic use
Child
Combined Modality Therapy / methods*
Deep Brain Stimulation / methods*
Drug Resistant Epilepsy / etiology
Drug Resistant Epilepsy / therapy*
Humans
Interleukin 1 Receptor Antagonist Protein / therapeutic use*
Male
Status Epilepticus / etiology
Status Epilepticus / therapy
Thalamic Nuclei / physiology
Treatment Outcome

Substances

Anticonvulsants
Interleukin 1 Receptor Antagonist Protein