title: Disorders of early language development in Dravet syndrome.
abstract: The aim of this study was to investigate language disorders prospectively in patients with Dravet syndrome (DS) during the first years of life in order to identify their features and possibly the underlying mechanisms of the disease. At the Child Neurology Unit of Catholic University in Rome (Italy), thirteen patients with typical findings of DS were enrolled in the study. Full clinical observations, including neurological examination and long-term EEG monitoring, were prospectively and serially performed until a mean of 6years of age (range: 4years to 7years and 8months). The epileptic history was also collected in each case. In particular, developmental, cognitive, and detailed language assessments were performed with different tests according to the age of the patient. In addition to cognitive decline, characteristic language impairment was also found with a relative preservation of receptive abilities (comprehension) and a strong impairment of productive skills. This defect in sensorimotor verbal processing integration is discussed to highlight the possible mechanisms underlying cognitive decline.
authors.affiliation: Child Neurology and Psychiatry, Catholic University, Rome, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Chieffo
authors.affiliation: Child Neurology and Psychiatry, Catholic University, Rome, Italy.
authors.forename: Domenica
authors.initials: D
authors.lastname: Battaglia
authors.affiliation: Child Neurology and Psychiatry, Catholic University, Rome, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Lucibello
authors.affiliation: Child Neurology and Psychiatry, Catholic University, Rome, Italy.
authors.forename: Maria Luigia
authors.initials: ML
authors.lastname: Gambardella
authors.affiliation: Child Neurology and Psychiatry, Catholic University, Rome, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Moriconi
authors.affiliation: Child Neurology and Psychiatry, Catholic University, Rome, Italy.
authors.forename: Gloria
authors.initials: G
authors.lastname: Ferrantini
authors.affiliation: Child Neurology and Psychiatry, Catholic University, Rome, Italy.
authors.forename: Giuseppina
authors.initials: G
authors.lastname: Leo
authors.affiliation: Child Neurology and Psychiatry, Catholic University, Rome, Italy.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Dravet
authors.affiliation: Child Neurology and Psychiatry, Catholic University, Rome, Italy.
authors.forename: Eugenio
authors.initials: E
authors.lastname: Mercuri
authors.affiliation: Child Neurology and Psychiatry, Catholic University, Rome, Italy. Electronic address: fguzzetta@rm.unicatt.it.
authors.forename: Francesco
authors.initials: F
authors.lastname: Guzzetta
mesh_terms: D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000150:complications*; D032882:Comprehension; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000150:complications*; D005260:Female; D006801:Humans; D007804:Language Development; D007805:Language Development Disorders / Q000150:complications*; D008297:Male; D009460:Neurologic Examination; D009483:Neuropsychological Tests
keywords: Cognitive decline; Dravet syndrome; Language developmental disorders