Abstract
Background
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.
Methods
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.
Results
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.
Conclusion
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.
Keywords
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Article info
Publication history
Published online: February 23, 2017
Accepted:
February 16,
2017
Received:
January 9,
2017
Identification
Copyright
© 2017 Published by Elsevier Inc.
