Original Article| Volume 71, P18-23.e2, June 2017

Cannabidiol Treatment for Refractory Seizures in Sturge-Weber Syndrome



      Sturge-Weber syndrome results in leptomeningeal vascular malformations, medically refractory epilepsy, stroke(s), and cognitive impairments. Cannabidiol, a cannabinoid without psychoactive properties, has been demonstrated in preclinical models to possibly have anticonvulsant, antioxidant, and neuroprotective actions.


      Five subjects with Sturge-Weber syndrome brain involvement and treatment-resistant epilepsy were enrolled. Motor seizure frequency, quality of life, and adverse events were recorded from the eighth week of the pretreatment period, eight weeks after starting maintenance dose (week 14), and the most recent visit.


      Four subjects had data through week 14, one of whom initially withdrew for lack of efficacy but because of other benefits re-enrolled with a lower dose. Two subjects at week 14 and three subjects with bilateral brain involvement had at the last visit a greater than 50% seizure reduction, reported an improved quality of life, and remained on cannabidiol 63-80 weeks after starting the drug. Three subjects reported mild side effects considered related to cannabidiol.


      This study suggests that cannabidiol may be well tolerated as adjunctive medication for seizure management and provides initial data supporting further study of cannabidiol in individuals with Sturge-Weber syndrome.


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        • Shirley M.D.
        • Tang H.
        • Gallione C.J.
        • et al.
        Sturge-Weber syndrome and port wine stains caused by somatic mutation in GNAQ.
        N Engl J Med. 2013; 368: 1971-1979
        • Comi A.M.
        Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-Weber syndrome.
        Neurologist. 2011; 17: 179-184
        • Comi A.M.
        Current therapeutic options in Sturge-Weber syndrome.
        Semin Pediatr Neurol. 2015; 22: 295-301
        • Eivazi B.
        • Roessler M.
        • Pfützner W.
        • et al.
        Port-wine stains are more than skin-deep! Expanding the spectrum of extracutaneous manifestations of nevi flammei of the head and neck.
        Eur J Dermatol. 2012; 22: 246-251
        • Sullivan T.J.
        • Clarke M.P.
        • Morin J.D.
        The ocular manifestations of the Sturge-Weber syndrome.
        J Pediatr Ophthalmol Strabismus. 1992; 29: 349-356
        • Comati A.
        • Beck H.
        • Halliday W.
        • et al.
        Upregulation of hypoxia-inducible factor (HIF)-1alpha and HIF-2alpha in leptomeningeal vascular malformations of Sturge-Weber syndrome.
        J Neuropathol Exp Neurol. 2007; 66: 86-97
        • Alkonyi B.
        • Chugani H.T.
        • Karia S.
        • Behen M.E.
        • Juhász C.
        Clinical outcomes in bilateral Sturge-Weber syndrome.
        Pediatr Neurol. 2011; 44: 443-449
        • Leo A.
        • Russo E.
        • Elia M.
        Cannabidiol and epilepsy: Rationale and therapeutic potential.
        Pharmacol Res. 2016; 107: 85-92
        • Devinsky O.
        • Marsh E.
        • Friedman D.
        • et al.
        Cannabidiol in patients with treatment-resistant epilepsy: an open-label interventional trial.
        Lancet Neurol. 2016; 15: 270-278
        • England T.J.
        • Hind W.H.
        • Rasid N.A.
        • O'Sullivan S.E.
        Cannabinoids in experimental stroke: a systematic review and meta-analysis.
        J Cereb Blood Flow Metab. 2015; 35: 348-358
        • Stanley C.P.
        • Hind W.H.
        • Tufarelli C.
        • O'Sullivan S.E.
        Cannabidiol causes endothelium-dependent vasorelaxation of human mesenteric arteries via CB1 activation.
        Cardiovasc Res. 2015; 107: 568-578
        • O'Sullivan S.E.
        • Sun Y.
        • Bennett A.J.
        • Randall M.D.
        • Kendall D.A.
        Time-dependent vascular actions of cannabidiol in the rat aorta.
        Eur J Pharmacol. 2009; 612: 61-68
        • Kelley T.M.
        • Hatfield L.A.
        • Lin D.D.
        • et al.
        Quantitative analysis of cerebral cortical atrophy and correlation with clinical severity in unilateral Sturge-Weber syndrome.
        J Child Neurol. 2005; 20: 867-870
        • Bhattacharyya S.
        • Atakan Z.
        • Martin-Santos R.
        • et al.
        Neural mechanisms for the cannabinoid modulation of cognition and affect in man: a critical review of neuroimaging studies.
        Curr Pharm Des. 2012; 18: 5045-5054
        • Kluger B.
        • Triolo P.
        • Jones W.
        • et al.
        The therapeutic potential of cannabinoids for movement disorders.
        Mov Disord. 2015; 30: 313-327