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 6 years of age (range: 4 years to 7 years and 8 months). 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.

Chieffo, D. P., Battaglia, D. I., Lucibello, S., Gambardella, M. L., Moriconi, F., Ferrantini, G., Leo, G., Dravet, C., Mercuri, E. M., Guzzetta, F., Disorders of early language development in Dravet syndrome, <<EPILEPSY & BEHAVIOR>>, 2016; 54 (1): 30-33. [doi:10.1016/j.yebeh.2015.10.027] [http://hdl.handle.net/10807/92426]

Disorders of early language development in Dravet syndrome

Chieffo, Daniela Pia
Primo
;
Battaglia, Domenica Immacolata
Secondo
;
Lucibello, Simona;Gambardella, Maria Luigia;Moriconi, Federica;Ferrantini, Gloria;Leo, Giuseppina;Dravet, Charlotte;Mercuri, Eugenio Maria
Penultimo
;
Guzzetta, Francesco
2016

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 6 years of age (range: 4 years to 7 years and 8 months). 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.
Inglese
Chieffo, D. P., Battaglia, D. I., Lucibello, S., Gambardella, M. L., Moriconi, F., Ferrantini, G., Leo, G., Dravet, C., Mercuri, E. M., Guzzetta, F., Disorders of early language development in Dravet syndrome, <<EPILEPSY & BEHAVIOR>>, 2016; 54 (1): 30-33. [doi:10.1016/j.yebeh.2015.10.027] [http://hdl.handle.net/10807/92426]
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/10807/92426
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