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

TY - JOUR

T1 - Centromedian thalamic nuclei deep brain stimulation and Anakinra treatment for FIRES – Two different outcomes

AU - Sa, Mario

AU - Singh, Rinki

AU - Pujar, Suresh

AU - D'arco, Felice

AU - Desai, Nivedita

AU - Eltze, Christin

AU - Hughes, Elaine

AU - Al Obaidi, Muthana

AU - Eleftheriou, Despina

AU - Tisdall, Martin

AU - Selway, Richard

AU - Cross, J. Helen

AU - Kaliakatsos, Marios

AU - Valentin, Antonio

PY - 2019/9/1

Y1 - 2019/9/1

N2 - 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.

AB - 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.

U2 - 10.1016/j.ejpn.2019.08.001

DO - 10.1016/j.ejpn.2019.08.001

M3 - Article

SN - 1090-3798

VL - 23

SP - 749

EP - 754

JO - European Journal of Paediatric Neurology

JF - European Journal of Paediatric Neurology

IS - 5

ER -