Seizure and EEG patterns in Wolf-Hirschhorn (4p-) syndrome.
Wolf-Hirschhorn syndrome (WHS) is a well-characterized chromosomal disorder that occurs due to partial deletion of the short arm of chromosome 4 (4p-). Although, about 300 cases have been reported to date, limited data are available on electroclinical findings. Information given to parents at the time of diagnosis tends to be skewed to the extreme negative. To delineate the natural history of seizures and EEG patterns in WHS, and obtain better information on diagnosis or outcome in a clinical setting, we reviewed the available literature on electroclinical findings of WHS. 4p- syndrome is characterized by distinctive seizure and EEG patterns that facilitate the early diagnosis and management of such patients.
Battaglia
Agatino
A
Stella Maris Clinical Research Institute for Child and Adolescent Neurology and Psychiatry, via dei Giaicnti 2-56018 Calambrone, Pisa, Italy. abattaglia@inpe.unipi.it
Carey
John C
JC
D000015:Abnormalities, Multiple / Q000503:physiopathology*; D001921:Brain / Q000503:physiopathology*; D002675:Child, Preschool; D002872:Chromosome Deletion; D025063:Chromosome Disorders / Q000503:physiopathology*; D002894:Chromosomes, Human, Pair 4 / Q000235:genetics*; D004569:Electroencephalography; D006801:Humans; D007223:Infant; D012640:Seizures / Q000503:physiopathology*