TY - JOUR
T1 - Lennox-Gastaut syndrome in adulthood
T2 - Clinical and EEG features
AU - Ferlazzo, Edoardo
AU - Nikaronova, Marina
AU - Italiano, Domenico
AU - Bureau, Michelle
AU - Dravet, Charlotte
AU - Calarese, Tiziana
AU - Viallat, Danielle
AU - Kölmel, Margarethe
AU - Bramanti, Placido
AU - De Santi, Lorenzo
AU - Genton, Pierre
PY - 2010/5
Y1 - 2010/5
N2 - Purpose: We performed a retrospective study to investigate seizure, EEG, social and cognitive outcome in adult LGS subjects. Methods: We retrospectively evaluated 27 LGS patients aged 40-59 years. We assessed in particular the evolution of different seizure types and EEG findings, as well as cognitive and social outcome. Results: During the early stages of the disease, all patients presented tonic seizures (TS) during wakefulness and sleep, 20/27 had atypical absences (AA), more rarely other seizure types. EEG showed slow background activity in 21/27 patients, diffuse slow spike-wave discharges (DSSW) during wakefulness in 22/27, and bursts of diffuse fast rhythms (DFR) in sleep in all patients. At last observation, 11 patients only had TS during wakefulness, but all still presented TS during sleep; AA persisted in 6 patients. EEG showed normal BA in 12/27 patients; only 7/27 still presented DSSW. On the contrary, sleep EEG showed the persistence of DFR in all. A moderate to severe cognitive impairment was observed in 26/27 patients. Conclusions: In adult LGS patients TS during sleep remain the major seizure type; moreover, a standard waking EEG may be normal. Thus, polysomnography represents the most important mean of investigation also in adult LGS patients.
AB - Purpose: We performed a retrospective study to investigate seizure, EEG, social and cognitive outcome in adult LGS subjects. Methods: We retrospectively evaluated 27 LGS patients aged 40-59 years. We assessed in particular the evolution of different seizure types and EEG findings, as well as cognitive and social outcome. Results: During the early stages of the disease, all patients presented tonic seizures (TS) during wakefulness and sleep, 20/27 had atypical absences (AA), more rarely other seizure types. EEG showed slow background activity in 21/27 patients, diffuse slow spike-wave discharges (DSSW) during wakefulness in 22/27, and bursts of diffuse fast rhythms (DFR) in sleep in all patients. At last observation, 11 patients only had TS during wakefulness, but all still presented TS during sleep; AA persisted in 6 patients. EEG showed normal BA in 12/27 patients; only 7/27 still presented DSSW. On the contrary, sleep EEG showed the persistence of DFR in all. A moderate to severe cognitive impairment was observed in 26/27 patients. Conclusions: In adult LGS patients TS during sleep remain the major seizure type; moreover, a standard waking EEG may be normal. Thus, polysomnography represents the most important mean of investigation also in adult LGS patients.
KW - Adults
KW - EEG
KW - Lennox-Gastaut syndrome
KW - Seizures
UR - http://www.scopus.com/inward/record.url?scp=77951297434&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77951297434&partnerID=8YFLogxK
U2 - 10.1016/j.eplepsyres.2010.01.012
DO - 10.1016/j.eplepsyres.2010.01.012
M3 - Article
C2 - 20149600
AN - SCOPUS:77951297434
VL - 89
SP - 271
EP - 277
JO - Epilepsy Research
JF - Epilepsy Research
SN - 0920-1211
IS - 2-3
ER -