Open AccessOrigins of Epilepsy in Fragile X Syndrome
Author(s) -
Hagerman Paul J.,
Stafstrom Carl E.
Publication year - 2009
Publication title -
epilepsy currents
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.415
H-Index - 22
eISSN - 1535-7511
pISSN - 1535-7597
DOI - 10.1111/j.1535-7511.2009.01309.x
Subject(s) - fragile x syndrome , medicine , epilepsy , seizure disorders , fragile x , autism spectrum disorder , psychiatry , autism , neuroscience , psychology , genetics , biology , gene
Fragile X syndrome is the leading heritable form of cognitive impairment and the leading known monogenic disorder associated with autism. Roughly one‐quarter of children with this disorder have seizures, most of which are relatively benign and are resolved beyond childhood. Because of the prevalence of fragile X syndrome, numerous animal models have been developed and electrophysiological studies have taken place to investigate its pathogenesis. The investigations have yielded a wealth of information regarding the synaptic dysfunction that underlies the hyperexcitability and epileptiform features associated with this disorder.