Cognitive, adaptive, and behavioral effects of adjunctive rufinamide in Lennox–Gastaut syndrome: A prospective observational clinical study
Autor: | Claudio Zucca, Michela Sole, Pasquale Striano, Francesca Felicia Operto, Grazia Maria Giovanna Pastorino, Alfonso Marrelli, Alberto Verrotti, Santina Città, Roberta Ciuffini, Giangennaro Coppola, Maurizio Elia, Valentina Manfredi |
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Jazyk: | angličtina |
Předmět: |
Adult
Pediatrics medicine.medical_specialty Adolescent CBCL Rufinamide Cognitive profile 03 medical and health sciences Behavioral Neuroscience Epilepsy Young Adult 0302 clinical medicine Cognition Medicine Humans 030212 general & internal medicine Prospective Studies Child Behavior Checklist Child Seizure types business.industry Lennox Gastaut Syndrome Lennox–Gastaut syndrome Neuropsychology Middle Aged Triazoles medicine.disease Neurology Adjunctive treatment Anticonvulsants Neurology (clinical) business 030217 neurology & neurosurgery medicine.drug |
Popis: | Introduction Lennox–Gastaut syndrome (LGS) is a severe pediatric epilepsy syndrome characterized by multiple drug-resistant seizure types. Children with LGS usually experience cognitive regression, and LGS is almost always associated with moderate to severe cognitive impairment. Rufinamide (RFM) was approved by the European Medicines Agency in 2007 for the adjunctive treatment of seizures associated with LGS in patients ≥ 4 years of age. The primary objective of our study was to assess cognitive, adaptive, and behavior functioning of patients with LGS after 12 months of RFM therapy. Methods This was an observational, multicenter, prospective study involving 16 patients diagnosed with LGS aged between 7 and 58 years (mean = 22 ± 16.3). Fourteen of 16 patients were already on therapy with 3 antiseizure drugs and 2/16 with 4 antiseizure drugs; RFM has been added with 100 mg/week increments up to a dose of 300–2400 mg/day. The participants and their parents underwent a neuropsychological evaluation for the assessment of intellectual, adaptive, and emotional/behavioral functioning (Leiter International Performance Scale-Revised (LEITER-R), Vineland, and Child Behavior CheckList (CBCL), respectively) before the RFM introduction (baseline) and 12 months after the RFM therapy (T2). Physical and neurological examination, electroencephalography (EEG) recording, seizure type and frequency, and adverse reactions were also considered. Results After 12 months, the total intelligence quotient (IQ) assessed by LEITER-R did not show statistical significant changes, such as there were no statistically significant changes in adaptive functions, assessed by Vineland. Furthermore, there were no statistically significant changes in internalizing and externalizing problems assessed by CBCL. Conclusion Adjunctive treatment with RFM did not negatively affect cognitive, adaptive function, and emotional profile in patients with LGS after 1 year of follow-up. |
Databáze: | OpenAIRE |
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