Myoclonic super-refractory status epilepticus with favourable evolution in a teenager with FIRES: Is the association of vagus nerve stimulation and cannabidiol effective?

Bonardi, Claudia; Furlanis, Giulia; Toldo, Irene; Guarrera, Brando; Luisi, Concetta; Pettenazzo, Andrea; Nosadini, Margherita; Boniver, Clementina; Sartori, Stefano; Landi, Andrea

doi:10.1016/j.braindev.2023.01.004

Cited by 6 publications

(9 citation statements)

References 12 publications

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“…However, it is likely that several, not yet completely understood, mechanisms underlie the action of the endocannabinoid system in modulating epileptogenesis. 7 Moreover, given the complex pathogenesis of the disease and its refractoriness to drugs, critically ill patients with FIRES undergo several therapies in the acute phase of the disease and the definition of the exact extent to which each therapy contributes to FIRES resolution is challenging. Both cases 1 and 2 started KD about 2 weeks before the administration of CBD.…”

Section: Discussionmentioning

confidence: 99%

“…It is, therefore, conceivable that CBD may have helped in counteracting the proepileptogenic mechanisms induced by both the initial immune dysregulation and the persistence of the SE itself. However, it is likely that several, not yet completely understood, mechanisms underlie the action of the endocannabinoid system in modulating epileptogenesis 7 …”

Section: Discussionmentioning

confidence: 99%

“…Other than the early administration of traditional ASMs and immune modulatory drugs, the use of cannabidiol (CBD) has been suggested at any time during the course of the disease as an adjunctive treatment to modulate epileptogenesis. 3,5,6,7 Nevertheless, literature evidence about its use in the acute phase of FIRES is poor and very few cases have been reported with uncertainty regarding the efficacy. Long-term outcome data are also lacking.…”

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confidence: 99%

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Cannabidiol in the acute phase of febrile infection‐related epilepsy syndrome (FIRES)

et al. 2023

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Febrile infection‐related epilepsy syndrome (FIRES) is a prolonged refractory status epilepticus (SE) that develops among healthy individuals after a febrile infection. FIRES treatment is challenging due to its poor response to antiseizure medications (ASMs) and anesthetic drugs. The use of cannabidiol (CBD) as an adjunctive treatment has been suggested, albeit data about its role in the acute phase is lacking. This report describes the use of purified CBD in the acute phase of two pediatric cases of FIRES and their long‐term outcome. Both children were treated with several ASMs, immunomodulators, anesthetics, and nonpharmacological treatment (ketogenic diet). CBD was administered, as an adjunctive treatment, through nasogastric tube about 30 days after onset. SE resolved within 3 days of reaching the target dose and both were seizure‐free for 1 year after. Although it is difficult to define the extent to which each previous therapy contributed to recovery, in both cases CBD therapy was a turning point, reinforcing its potential role as add‐on treatment in the acute phase of FIRES.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Cannabidiol in the acute phase of febrile infection‐related epilepsy syndrome (FIRES)

et al. 2023

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“…Five patients were started on the ketogenic diet ( 16 , 17 , 19 ), without aborting status epilepticus, although adequacy of ketosis was never documented. In nine cases, a trial of immunosuppression was described, with most patients undergoing a combination regime of pulsed steroids [six cases ( 6 , 16 , 18 – 20 , 22 )], followed by ivIg [six cases ( 6 , 16 , 18 – 20 , 22 )], Plasma exchange [four cases ( 6 , 16 , 22 , 25 )] or Rituximab [two cases ( 19 , 25 )].…”

Section: Reported Cases Of Vns Use In Norse/firesmentioning

confidence: 99%

“…Resistance to drug therapy is a major hurdle in managing this group of patients and there is urgent need to develop new treatment approaches. Functional disability is present in up to two thirds of NORSE survivors and subsequent chronic epilepsy is the norm ( 2 – 5 ), but individual reports and our own experience indicates that some people do have good cognitive and functional outcomes, even after prolonged status epilepticus ( 6 ). Whether desynchronizing networks by vagal nerve stimulation (VNS) may improve seizure control remains an unanswered question.…”

Section: Introductionmentioning

confidence: 99%

Perspective: Vagal nerve stimulation in the treatment of new-onset refractory status epilepticus

Ritter

Selway

2023

Front. Neurol.

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IntroductionResistance to drug therapy is a major hurdle in new-onset refractory status epilepticus (NORSE) treatment and there is urgent need to develop new treatment approaches. Non-drug approaches such as neuromodulation offer significant benefits and should be investigated as new adjunct treatment modalities. An important unanswered question is whether desynchronizing networks by vagal nerve stimulation (VNS) may improve seizure control in NORSE patients.Main textWe present a summary of published NORSE cases treated with VNS and our own data, discuss possible mechanisms of action, review VNS implantation timing, stimulation setting titration protocols and outcomes. Further, we propose avenues for future research.DiscussionWe advocate for consideration of VNS for NORSE both in early and late stages of the presentation and hypothesize a possible additional benefit from implantation in the acute phase of the disease. This should be pursued in the context of a clinical trial, harmonizing inclusion criteria, accuracy of documentation and treatment protocols. A study planned within our UK-wide NORSE-UK network will answer the question if VNS may confer benefits in aborting unremitting status epilepticus, modulate ictogenesis and reduce long-term chronic seizure burden.

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Responsive neurostimulation as a therapy for epilepsy following new-onset refractory status epilepticus: Case series and review of the literature

Oliger,

Nerison,

Tan

et al. 2024

Clinical Neurophysiology

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Myoclonic super-refractory status epilepticus with favourable evolution in a teenager with FIRES: Is the association of vagus nerve stimulation and cannabidiol effective?

Cited by 6 publications

References 12 publications

Cannabidiol in the acute phase of febrile infection‐related epilepsy syndrome (FIRES)

Cannabidiol in the acute phase of febrile infection‐related epilepsy syndrome (FIRES)

Perspective: Vagal nerve stimulation in the treatment of new-onset refractory status epilepticus

Responsive neurostimulation as a therapy for epilepsy following new-onset refractory status epilepticus: Case series and review of the literature

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