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(Abst. 2.164), 2015

Cannabadiol in Genetic Refractive Epilepsy in Dravet and Non-Dravet Cases
Authors: Michael Chez
Content: Rationale: Cannabadiol ( CBD) has been explored in drug resistant childhood epilepsy, especially in Dravet Syndrome. Expanded access under FDA and DEA approval and collaboration with GW Pharma has led to the ability to offer compassionate access for some patients. However defined use in Dravet and non-Dravet genetic subtypes is still also evolving . These 3 cases are examples of response in SCN1A, GABR3 and SCN2A cases,Methods: The Expanded Access program enabled 3 patients on CBD to increase from 5mg/kg to 25mg/kg per day, GW Pharma provided 100mg/ml CBD preparation (Epiodiolex). Identification of refractory patients in our practice showed the following: 12year old female with SCN2A and > 120 seizures/ month of sleep-related tonic type; 21 year old female with GABR3 and >180 seizures/month; 3.5 year old male SCNA1 variant with 1000-2000 head drop / >16 tonic seizures/month. Approval from Sutter System Central IRB was obtained. Baseline seizure count for 4 weeks was followed by weekly titration to maximal effect or side effects.Results: All 3 patients responded dramatically to CBD. At 5mg/kg response was seen in the GABR3 case, and titration up to 25mg/kg/day was tolerated. The SCN1A case dramatically improved and after 10mg/kg up through 25mg/kg/day improved each step, The SCN2Apatient responded after 10mg/kg per to maximal 25mg/kg/day. Maximal dose was tolerated in all cases. Because of CBD affect on other drug metabolism, doses of clonazapam were lowered in the Dravet case from 2mg TID to 1mg TID for ataxia, Clobazam (from 40mg qhs to 30mg, and felbamate ;owered from 1600mg to 1200mg in SCN2A case for dizziness/fatigue. The GABR3 required lamotrigine lowered from 600mg qhs to 500mg. All tolerated these changes. Noted seizure reductions on maximal 25mg/kg/day CBD were: SCN2A pre 120/month to post < 30 sleep onset aura only of body stiffening( no visible events), SCN1A showed head drops reduced from > 1000/month to 30/month and tonic 20 to 4/month, GABR3 reduced from >130/month to < 30 shorter sleep onset only events. .EEG results pending in all patients post CBD, All patients reported improved moods, improved social or cognitive skills, and all tolerated initial dose reductions of some of their concurrent chronic AED. There were no serious adverse events. Patients’ results are at 8 weeks post initiation CBD. .Conclusions: This clinical use of CBD( Epidiolex) in 3 patients with different intractable epilepsy from different genetic subtypes shows great efficacy in Dravet variant SCN1A, SCN2A, and GABR3. All patients had EEG signs of tonic or atonic and other mixed seizure types. This suggests that in addition to SCN1A, other genetic subtypes of Lennox -Gastaut seizures with tonic or ataonic and mixed seizure types seem to do well on a pure CBD product. Further collection of genetic subtypes who meet this criteria may expand knowledge of which patients the use of CBD may be clinically more efficatious.EEG data before and after treatment also will help us understand the efficacy of CBD. Observed cognitive, attention, and mood improvements also indicate braod affect of the CBD compound.