←Epilepsy
Study register · detail Fallserie (Open-Label, erweiterter Zugang, 5 Zentren) · Epilepsy · 2017

Cannabidiol as a Potential Treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES) in the Acute and Chronic Phases.

Gofshteyn et al. ·Journal of Child Neurology
Mixed GRADE Very low 150 citations
Samplen = 7 Pat.
EndpointSeizure frequency and duration
Blindingn.a.
DesignFallserie (Open-Label, erweiterter Zugang, 5 Zentren)
Cannabinoidcbd
Routeoral
Key finding

Cannabidiol reduced seizure frequency and duration in 6 of 7 children, however one patient died of treatment-associated multi-organ failure.

Summary

n=7 children with FIRES (febrile infection-related epilepsy syndrome) from 5 centres, CBD (Epidiolex) in the acute or chronic phase. 6 of 7 patients (86%) showed improvement in seizure frequency and duration. On average 4 antiepileptic drugs discontinued. Currently: 5 patients able to walk, 4 able to speak. 1 patient died of multi-organ failure (isoflurane-associated, not CBD-related). Case series in an ultra-rare, otherwise treatment-refractory epilepsy.

P
PopulationChildren with FIRES (Febrile Infection-Related Epilepsy Syndrome), treatment-refractory after multiple antiepileptic drugs, n=7, from 5 centres
I
InterventionCannabidiol (Epidiolex, GW Pharma), oral, as part of an emergency or expanded access protocol in the acute or chronic phase
O
OutcomeIn 6 of 7 patients improvement in seizure frequency and duration; on average 4 antiepileptic drugs could be reduced; 1 patient died of multi-organ failure (isoflurane-associated)
Confidence in the evidence
Very low

The lowest GRADE level, the effect estimate remains uncertain.

Downgraded for
Risk of biasImprecision
Quality profile
Sample size ★★★★★
Blinding —
Effect size Mixed
Citations / year ★★★★★
Authors
Gofshteyn JS, Wilfong A, Devinsky O, Bluvstein J, Charuta J, Ciliberto MA, Laux L, Marsh ED.
DOI 10.1177/0883073816669450↗
Design: Fallserie (Open-Label, erweiterter Zugang, 5 Zentren)
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Abstract
Febrile infection-related epilepsy syndrome (FIRES) is a devastating epilepsy affecting normal children after a febrile illness. FIRES presents with an acute phase with super-refractory status epilepticus and all patients progress to a chronic phase with persistent refractory epilepsy. The typical outcome is severe encephalopathy or death. The authors present 7 children from 5 centers with FIRES who had not responded to antiepileptic drugs or other therapies who were given cannabadiol (Epidiolex, GW Pharma) on emergency or expanded investigational protocols in either the acute or chronic phase of illness. After starting cannabidiol, 6 of 7 patients' seizures improved in frequency and duration. One patient died due to multiorgan failure secondary to isoflourane. An average of 4 antiepileptic drugs were weaned. Currently 5 subjects are ambulatory, 1 walks with assistance, and 4 are verbal. While this is an open-label case series, the authors add cannabidiol as a possible treatment for FIRES.
Indication
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The impediment to action advances action. — Marcus Aurelius