Peculiare presentazione clinica della sindrome di Lennox-Gastaut in pazienti con trisomia 21

Translated title of the contribution: Peculiar clinical features of Lennox-Gastaut syndrome in trisomy 21 patients

D. Italiano, E. Ferlazzo, C. K. Adjien, R. Guerrini, M. Elia, P. Striano, A. Crespel, T. Calarese, P. Bramanti, P. Di Bella, P. Gelisse, P. Genton

Research output: Contribution to journalArticle

Abstract

Lennox-Gastaut syndrome (LGS) has been rarely and incompletely described in subjects with Down syndrome (DS). We retrospectively collected the clinical and EEG data of 13 consecutive patients with LGS and DS referred to 4 epilepsy centres over the last 20 years. The mean age at onset was 9.09 years (range 5-16). There were no patients with seizure onset before the age of 5 years, 5 patients (38%) with seizure onset between 5 and 8 years,while 8 patients (61%) had seizures onset after age 8.Nine patients (69%) had unambiguous reflex seizures mostly precipitated by sudden unexpected sensory stimulations, usually preceding or accompanying the onset of full-blown LGS picture. Interictal and ictal EEG findings were typical for LGS.All patients were drugresistant. Our data suggest that patients with DS may present a peculiar LGS characterized by late onset and high occurrence of reflex seizures.

Original languageItalian
Pages (from-to)167-168
Number of pages2
JournalBollettino - Lega Italiana contro l'Epilessia
Issue number138
Publication statusPublished - 2008

Fingerprint

Down Syndrome
Seizures
Age of Onset
Reflex
Electroencephalography
Lennox Gastaut Syndrome
Epilepsy
Stroke

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Peculiare presentazione clinica della sindrome di Lennox-Gastaut in pazienti con trisomia 21. / Italiano, D.; Ferlazzo, E.; Adjien, C. K.; Guerrini, R.; Elia, M.; Striano, P.; Crespel, A.; Calarese, T.; Bramanti, P.; Di Bella, P.; Gelisse, P.; Genton, P.

In: Bollettino - Lega Italiana contro l'Epilessia, No. 138, 2008, p. 167-168.

Research output: Contribution to journalArticle

Italiano, D, Ferlazzo, E, Adjien, CK, Guerrini, R, Elia, M, Striano, P, Crespel, A, Calarese, T, Bramanti, P, Di Bella, P, Gelisse, P & Genton, P 2008, 'Peculiare presentazione clinica della sindrome di Lennox-Gastaut in pazienti con trisomia 21', Bollettino - Lega Italiana contro l'Epilessia, no. 138, pp. 167-168.
Italiano, D. ; Ferlazzo, E. ; Adjien, C. K. ; Guerrini, R. ; Elia, M. ; Striano, P. ; Crespel, A. ; Calarese, T. ; Bramanti, P. ; Di Bella, P. ; Gelisse, P. ; Genton, P. / Peculiare presentazione clinica della sindrome di Lennox-Gastaut in pazienti con trisomia 21. In: Bollettino - Lega Italiana contro l'Epilessia. 2008 ; No. 138. pp. 167-168.
@article{552cbeb6f0e84ca482521d210cb2c2c9,
title = "Peculiare presentazione clinica della sindrome di Lennox-Gastaut in pazienti con trisomia 21",
abstract = "Lennox-Gastaut syndrome (LGS) has been rarely and incompletely described in subjects with Down syndrome (DS). We retrospectively collected the clinical and EEG data of 13 consecutive patients with LGS and DS referred to 4 epilepsy centres over the last 20 years. The mean age at onset was 9.09 years (range 5-16). There were no patients with seizure onset before the age of 5 years, 5 patients (38{\%}) with seizure onset between 5 and 8 years,while 8 patients (61{\%}) had seizures onset after age 8.Nine patients (69{\%}) had unambiguous reflex seizures mostly precipitated by sudden unexpected sensory stimulations, usually preceding or accompanying the onset of full-blown LGS picture. Interictal and ictal EEG findings were typical for LGS.All patients were drugresistant. Our data suggest that patients with DS may present a peculiar LGS characterized by late onset and high occurrence of reflex seizures.",
keywords = "Down syndrome, Epilepsy, Lennox-Gastaut syndrome",
author = "D. Italiano and E. Ferlazzo and Adjien, {C. K.} and R. Guerrini and M. Elia and P. Striano and A. Crespel and T. Calarese and P. Bramanti and {Di Bella}, P. and P. Gelisse and P. Genton",
year = "2008",
language = "Italian",
pages = "167--168",
journal = "Bollettino - Lega Italiana contro l'Epilessia",
issn = "0394-560X",
publisher = "Lega Italiana contro l'Epilessia",
number = "138",

}

TY - JOUR

T1 - Peculiare presentazione clinica della sindrome di Lennox-Gastaut in pazienti con trisomia 21

AU - Italiano, D.

AU - Ferlazzo, E.

AU - Adjien, C. K.

AU - Guerrini, R.

AU - Elia, M.

AU - Striano, P.

AU - Crespel, A.

AU - Calarese, T.

AU - Bramanti, P.

AU - Di Bella, P.

AU - Gelisse, P.

AU - Genton, P.

PY - 2008

Y1 - 2008

N2 - Lennox-Gastaut syndrome (LGS) has been rarely and incompletely described in subjects with Down syndrome (DS). We retrospectively collected the clinical and EEG data of 13 consecutive patients with LGS and DS referred to 4 epilepsy centres over the last 20 years. The mean age at onset was 9.09 years (range 5-16). There were no patients with seizure onset before the age of 5 years, 5 patients (38%) with seizure onset between 5 and 8 years,while 8 patients (61%) had seizures onset after age 8.Nine patients (69%) had unambiguous reflex seizures mostly precipitated by sudden unexpected sensory stimulations, usually preceding or accompanying the onset of full-blown LGS picture. Interictal and ictal EEG findings were typical for LGS.All patients were drugresistant. Our data suggest that patients with DS may present a peculiar LGS characterized by late onset and high occurrence of reflex seizures.

AB - Lennox-Gastaut syndrome (LGS) has been rarely and incompletely described in subjects with Down syndrome (DS). We retrospectively collected the clinical and EEG data of 13 consecutive patients with LGS and DS referred to 4 epilepsy centres over the last 20 years. The mean age at onset was 9.09 years (range 5-16). There were no patients with seizure onset before the age of 5 years, 5 patients (38%) with seizure onset between 5 and 8 years,while 8 patients (61%) had seizures onset after age 8.Nine patients (69%) had unambiguous reflex seizures mostly precipitated by sudden unexpected sensory stimulations, usually preceding or accompanying the onset of full-blown LGS picture. Interictal and ictal EEG findings were typical for LGS.All patients were drugresistant. Our data suggest that patients with DS may present a peculiar LGS characterized by late onset and high occurrence of reflex seizures.

KW - Down syndrome

KW - Epilepsy

KW - Lennox-Gastaut syndrome

UR - http://www.scopus.com/inward/record.url?scp=77953116325&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77953116325&partnerID=8YFLogxK

M3 - Articolo

AN - SCOPUS:77953116325

SP - 167

EP - 168

JO - Bollettino - Lega Italiana contro l'Epilessia

JF - Bollettino - Lega Italiana contro l'Epilessia

SN - 0394-560X

IS - 138

ER -