Cannabidiol as an Adjunct Treatment for Children with Febrile Infection-Related Epilepsy Syndrome (FIRES) Reduces Seizure Frequency and Dependence on Multiple Anti-Epileptic Drugs.
Abstract number :
3.454
Submission category :
18. Case Studies
Year :
2018
Submission ID :
554695
Source :
www.aesnet.org
Presentation date :
12/3/2018 1:55:12 PM
Published date :
Nov 5, 2018, 18:00 PM
Authors :
Michael S. Myette, Kaiser Permanente Medical Center, Roseville, CA
Rationale: FIRES is a devastating condition which presents in previously healthy children as an encephalitis-like syndrome with fevers and seizures. It rapidly progresses to super-refractory status epilepticus. No infectious or autoimmune cause is identified. Over time, patients transition to a chronic phase, with diminished neurologic function and persistent refractory epilepsy, often with waxing and waning severity. Most patients have poor outcomes, often progressing to severe encephalopathy, a minimally conscious state, or death. Methods: We present 3 cases within a single health system in Northern California (Kaiser Permanente) of Children with FIRES who failed to respond to multiple anti-epileptic drugs and immunomodulators in the acute phase, but all of whom responded to cannabidiol (CBD) with significant reduction in seizure activity. Patients were treated with Epidiolex(R)TM a pharmaceutical formulation of highly purified cannabidiol containing >= 98% CBD w/w API with trace residual amounts of THC (maximum of 0.1% w/w API) and other plant components. Epidiolex was delivered enterally at a concentration of 100mg/ml in a sesame oil base. Results: Patients demonstrated a significant reduction in seizure frequency after 4 weeks of treatment with CBD. All patients maintained moderate to excellent control of their seizures 48 weeks after initiation of therapy. All patients were able to significantly reduce the number and dose of anti-epileptic drugs (AEDs) in their daily regimen after the indroduction of CBD. One patient has regained full consciousness and is communicating with the aid of a computer device, demonstrating high level abstract thinking and sparing of executive functions. One patient is able to follow simple commands, demonstrating sparing of receptive language. One patient remains in a minimally conscious state, but with better seizure control, and requiring fewer AEDs. Conclusions: In this subset of patients with FIRES, cannabidiol augmented seizure control, reduced the need for multiple other anti-epileptic drugs, and in some cases, facilitated a limited cognitive recovery. Funding: Funding: CBD was provided by GW Research Ltd. (Cambridge, UK)
