Lennox-Gastaut syndrome in adulthood : Long-term clinical follow-up of 38 patients and analysis of their recorded seizures
Academic Article
Publication Date:
2017
Citation:
Lennox-Gastaut syndrome in adulthood : Long-term clinical follow-up of 38 patients and analysis of their recorded seizures / A. Vignoli, O. Gaia, D.M. Giovanni, A. Peron, M.N. Savini, Z. Elena, V. Chiesa, F. La Briola, K. Turner, M.P. Canevini. - In: EPILEPSY & BEHAVIOR. - ISSN 1525-5050. - 77(2017 Dec), pp. 73-78. [10.1016/j.yebeh.2017.09.006]
abstract:
Lennox–Gastaut syndrome (LGS) is a severe epileptic encephalopathy with childhood onset that usually continues
through adolescence and into adulthood. In the long term, patients with this condition still have intractable
seizures, intellectual disability, behavioral problems, and physical comorbidities. The aimof this studywas to
describe the clinical and EEG characteristics of a group of adults with Lennox–Gastaut syndrome. We identified
38 (22 females, 16 males) patients with LGS older than age 18 years at their last evaluation, with mean age of
43.3 ± 10.6 years. Median follow-up was 14.4 years (range: 2–40).
All of our patients had 3 or more seizure types during their clinical history. The most prevalent seizure types at
follow-up were atypical absences (28/38), tonic (28/38), generalized tonic–clonic (17/38), focal (11/38), and
myoclonic seizures (9/38). All patients had drug-resistant seizures. Besides epilepsy, intellectual disability and
behavioral problems were prominent features. Surprisingly, paroxysmal nonepileptic seizures were reported
in 3 patients. Our observations confirm the poor outcome of Lennox–Gastaut syndrome through adulthood,
regardless of age at seizure onset, etiology, and history of previousWest syndrome.
through adolescence and into adulthood. In the long term, patients with this condition still have intractable
seizures, intellectual disability, behavioral problems, and physical comorbidities. The aimof this studywas to
describe the clinical and EEG characteristics of a group of adults with Lennox–Gastaut syndrome. We identified
38 (22 females, 16 males) patients with LGS older than age 18 years at their last evaluation, with mean age of
43.3 ± 10.6 years. Median follow-up was 14.4 years (range: 2–40).
All of our patients had 3 or more seizure types during their clinical history. The most prevalent seizure types at
follow-up were atypical absences (28/38), tonic (28/38), generalized tonic–clonic (17/38), focal (11/38), and
myoclonic seizures (9/38). All patients had drug-resistant seizures. Besides epilepsy, intellectual disability and
behavioral problems were prominent features. Surprisingly, paroxysmal nonepileptic seizures were reported
in 3 patients. Our observations confirm the poor outcome of Lennox–Gastaut syndrome through adulthood,
regardless of age at seizure onset, etiology, and history of previousWest syndrome.
IRIS type:
01 - Articolo su periodico
Keywords:
drug-resistance; Lennox-Gastaut syndrome; long-term evolution; seizures; video-EEG recordings; neurology; neurology (clinical); behavioral neuroscience
List of contributors:
A. Vignoli, O. Gaia, D.M. Giovanni, A. Peron, M.N. Savini, Z. Elena, V. Chiesa, F. La Briola, K. Turner, M.P. Canevini
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