Cannabidiol Treatment for Neurological, Cognitive, and Psychiatric Symptoms in Sturge-Weber Syndrome - 12/01/23
Abstract |
Background |
A prior drug trial of cannabidiol for treatment-resistant epilepsy in patients with Sturge-Weber syndrome (SWS), a rare neurovascular condition, implicated improvements in neurological, quality of life (QOL), neuropsychologic, psychiatric, and motor outcomes.
Methods |
Ten subjects with SWS brain involvement, controlled seizures, and cognitive impairments received study drug in this Johns Hopkins institutional review board-approved, open-label, prospective drug trial. Oral cannabidiol was taken for six months (dose ranged from 5 to 20 mg/kg/day). SWS neuroscore, port-wine birthmark score, QOL, and adverse events were recorded every four to 12 weeks. Neuropsychologic, psychiatric, and motor assessments were administered at baseline and six months’ follow-up. Most evaluations were conducted virtually due to the coronavirus disease 2019 pandemic.
Results |
Cannabidiol was generally well tolerated. Six subjects reported mild to moderate side effects related to study drug and continued on drug; one subject withdrew early due to moderate side effects. No seizures were reported. Significant improvements in SWS neuroscore, patient-reported QOL, anxiety and emotional regulation, and report of bimanual ability use were noted. Migraine QOL scores were high at baseline in these subjects, and remained high. Neuropsychologic and other QOL and motor outcomes remained stable, with some within-subject improvements noted.
Conclusions |
Further studies are needed to determine whether Epidiolex can improve quality of life and be beneficial for neurological, anxiety, and motor impairments in SWS independent of seizure control. Large multicentered studies are needed to extend these preliminary findings.
Le texte complet de cet article est disponible en PDF.Keywords : Sturge-Weber syndrome, Cannabidiol, Seizures, Anxiety
Plan
Declaration of Interest: A.M.C. is an inventor on a patent for cannabidiol in SWS; this study followed all conflict of interest management required by the Johns Hopkins Conflict of Interest Committee. T.R. is on NIH STTR Phase I project advisory board: Designing and Evaluating Outcome Assessment Software for Youth with Developmental Disabilities: and The Pediatric Evaluation of Disability Inventory-Patient Reported Outcome (PEDI-PRO); PEDI-PRO was derived from the PEDI-CAT used in this study. |
Vol 139
P. 24-34 - février 2023 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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