A case for cannabidiol in Wolf-Hirschhorn syndrome seizure management.

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A case for cannabidiol in Wolf-Hirschhorn syndrome seizure management.

Category : Epilepsia

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A case for cannabidiol in Wolf-Hirschhorn syndrome seizure management.

Am J Med Genet A. 2017 Feb;173(2):324-326

Authors: Ho KS, Wassman ER

Abstract
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.

PMID: 28102593 [PubMed – indexed for MEDLINE]

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