PMID- 34052628 OWN - NLM STAT- MEDLINE DCOM- 20210913 LR - 20210913 IS - 1532-2688 (Electronic) IS - 1059-1311 (Linking) VI - 91 DP - 2021 Oct TI - Two-center experience of cannabidiol use in adults with Dravet syndrome. PG - 5-8 LID - S1059-1311(21)00165-5 [pii] LID - 10.1016/j.seizure.2021.05.014 [doi] AB - We describe real-world experience with cannabidiol (CBD) in adults with Dravet Syndrome (DS) via GW Pharma early access programme at two UK neurology centres. Adults with genetically-confirmed DS had CBD added to existing therapy, titrated up to 20 mg/kg, as tolerated. The primary outcome measure was percentage reduction in convulsive seizures. Secondary outcome measures included changes in myoclonic seizures, and in cognition and quality of life as assessed by the Caregiver Global Impression of Change (CGIC), and incidence of adverse events (AEs). 18 adults (7 female; median age 27.5 years; range 20-51) were included. Median follow-up was 176 days. In one, another antiseizure drug, clobazam, was introduced during the programme. 3/17 (17.6%) had >30% reduction in convulsive seizures (range: 87.5-100%). AEs occurred in all, the most common being transaminitis (52.9%). Behavioural AEs led to discontinuation in 3/18 (16.7%), including a seizure-free responder. In 7/18, CBD was stopped due to lack of effect. 8/18 continue on treatment. Improvements in CGIC were reported in 41.2% and 47.1% by physicians and families, respectively. 17.6% achieved sufficient reduction in convulsive seizure frequency to qualify for NHS funding. AEs led to withdrawal in only 16.7%. Close monitoring and dose adjustments of other antiseizure drugs were necessary. CI - Copyright (c) 2021. Published by Elsevier Ltd. FAU - Silvennoinen, Katri AU - Silvennoinen K AD - Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, Box 29, Queen Square, London WC1N 3BG, UK; Chalfont Centre for Epilepsy, Chalfont St Peter, UK. FAU - Ritter, Laura Mantoan AU - Ritter LM AD - Clinical Neurosciences Department, King's College Hospital NHS Foundation Trust, Denmark Hill, London, UK. FAU - Nashef, Lina AU - Nashef L AD - Clinical Neurosciences Department, King's College Hospital NHS Foundation Trust, Denmark Hill, London, UK. FAU - Hudgell, Kirsty AU - Hudgell K AD - St. Elizabeth's Centre, Much Hadham, Herts, UK. FAU - Balestrini, Simona AU - Balestrini S AD - Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, Box 29, Queen Square, London WC1N 3BG, UK; Chalfont Centre for Epilepsy, Chalfont St Peter, UK. FAU - Sisodiya, Sanjay M AU - Sisodiya SM AD - Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, Box 29, Queen Square, London WC1N 3BG, UK; Chalfont Centre for Epilepsy, Chalfont St Peter, UK. FAU - Sidhu, Meneka K AU - Sidhu MK AD - Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, Box 29, Queen Square, London WC1N 3BG, UK; Chalfont Centre for Epilepsy, Chalfont St Peter, UK. LA - eng PT - Journal Article DEP - 20210524 PL - England TA - Seizure JT - Seizure JID - 9306979 RN - 0 (Anticonvulsants) RN - 19GBJ60SN5 (Cannabidiol) RN - 2MRO291B4U (Clobazam) SB - IM MH - Adult MH - Anticonvulsants/therapeutic use MH - *Cannabidiol/therapeutic use MH - Clobazam/therapeutic use MH - *Epilepsies, Myoclonic/drug therapy MH - Female MH - Humans MH - Middle Aged MH - Quality of Life MH - Young Adult OTO - NOTNLM OT - Antiseizure medication OT - Epilepsy OT - Seizures EDAT- 2021/05/31 06:00 MHDA- 2021/09/14 06:00 CRDT- 2021/05/30 20:54 PHST- 2021/03/17 00:00 [received] PHST- 2021/04/26 00:00 [revised] PHST- 2021/05/14 00:00 [accepted] PHST- 2021/05/31 06:00 [pubmed] PHST- 2021/09/14 06:00 [medline] PHST- 2021/05/30 20:54 [entrez] AID - S1059-1311(21)00165-5 [pii] AID - 10.1016/j.seizure.2021.05.014 [doi] PST - ppublish SO - Seizure. 2021 Oct;91:5-8. doi: 10.1016/j.seizure.2021.05.014. Epub 2021 May 24.