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 language | Italian |
|---|---|
| Pages (from-to) | 167-168 |
| Number of pages | 2 |
| Journal | Bollettino - Lega Italiana contro l'Epilessia |
| Issue number | 138 |
| Publication status | Published - 2008 |
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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 journal › Article
}
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 -