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Am J Med Genet A. 2017 Feb;173(2):324-326. doi: 10.1002/ajmg.a.37979. Epub 2016 Nov 7.

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

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Lineagen, Inc., Salt Lake City, Utah.
Department of Pediatrics, School of Medicine, University of Utah, Salt Lake City, Utah.


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.


Dravet syndrome; Wolf-Hirschhorn syndrome; cannabidiol; seizures

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