Journal
JOURNAL OF CHILD NEUROLOGY
Volume 32, Issue 1, Pages 35-40Publisher
SAGE PUBLICATIONS INC
DOI: 10.1177/0883073816669450
Keywords
pediatric epilepsy; cannabidiol; refractory status epilepticus; febrile infection-related epilepsy syndrome (FIRES)
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Funding
- GW pharmaceuticals (Salisbury, UK)
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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.
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