A case for cannabidiol in Wolf–Hirschhorn syndrome seizure management

Complex, and sometimes intractable, seizures affect the quality of life and cognitive development of over 90% of individuals with Wolf–Hirschhorn syndrome (WHS). Fine resolution genotype–phenotype mapping of the WHS locus recently identified a candidate gene whose probable function has led to insights into a mechanism connecting WHS seizures with those of Dravet syndrome, a distinct condition caused by mutations in SCN1A and SCN1B. In addition to this possible molecular mechanistic connection, these disorders' seizures share a strikingly similar constellation of features, including clinical presentation, seizure types, early age of onset, EEG pattern, and responses to specific anti-epileptic drugs. Based in part on these similarities, we suggest that a highly successful Phase III clinical trial of a formulation of cannabidiol for Dravet syndrome seizures may be directly translatable into possible benefits for WHS individuals with challenging seizure patterns. © 2016 Wiley Periodicals, Inc.

from Genetics via xlomafota13 on Inoreader http://ift.tt/2fgpMgh
via IFTTT