BACKGROUD: Drug-resistant epilepsy is the main risk factor for future intellectual disability in patients with tuberous sclerosis complex. Clinical epileptic seizures are often preceded by electroencephalographic changes, which provide an opportunity for preventive treatment. We evaluated the neuropsychologic and epilepsy outcomes at school age in children with tuberous sclerosis complex who received preventive antiepileptic treatment in infancy. METHODS: We performed a prospective, nonrandomized clinical trial with 14 infants diagnosed with tuberous sclerosis complex in whom serial electroencephalographic recordings were performed and preventive treatment with vigabatrin initiated when active epileptic discharges were detected. An age-matched control group consisted of 31 infants with tuberous sclerosis complex in whom treatment with vigabatrin was given only after onset of clinical seizures. Results of clinical assessment of epilepsy and cognitive outcomes were analyzed. RESULTS: All patients in the preventive group (n = 14) and 25 of 31 patients in the standard treatment group were followed through minimum age five years, median 8.8 and 8.0 years in the preventive and standard groups, respectively. The median intelligence quotient was 94 for the preventive group when compared with 46 for the standard group (P < 0.03). Seven of 14 patients (50%) in the preventive group never had a clinical seizure when compared with one of 25 patients (5%) in the standard treatment group (P = 0.001). CONCLUSIONS: This study provides evidence that preventive antiepileptic treatment in infants with tuberous sclerosis complex improves long-term epilepsy control and cognitive outcome at school age.
BACKGROUD: Drug-resistant epilepsy is the main risk factor for future intellectual disability in patients with tuberous sclerosis complex. Clinical epileptic seizures are often preceded by electroencephalographic changes, which provide an opportunity for preventive treatment. We evaluated the neuropsychologic and epilepsy outcomes at school age in children with tuberous sclerosis complex who received preventive antiepileptic treatment in infancy. METHODS: We performed a prospective, nonrandomized clinical trial with 14 infants diagnosed with tuberous sclerosis complex in whom serial electroencephalographic recordings were performed and preventive treatment with vigabatrin initiated when active epileptic discharges were detected. An age-matched control group consisted of 31 infants with tuberous sclerosis complex in whom treatment with vigabatrin was given only after onset of clinical seizures. Results of clinical assessment of epilepsy and cognitive outcomes were analyzed. RESULTS: All patients in the preventive group (n = 14) and 25 of 31 patients in the standard treatment group were followed through minimum age five years, median 8.8 and 8.0 years in the preventive and standard groups, respectively. The median intelligence quotient was 94 for the preventive group when compared with 46 for the standard group (P < 0.03). Seven of 14 patients (50%) in the preventive group never had a clinical seizure when compared with one of 25 patients (5%) in the standard treatment group (P = 0.001). CONCLUSIONS: This study provides evidence that preventive antiepileptic treatment in infants with tuberous sclerosis complex improves long-term epilepsy control and cognitive outcome at school age.
Authors: Leslie E Grayson; Jurriaan M Peters; Tarrant McPherson; Darcy A Krueger; Mustafa Sahin; Joyce Y Wu; Hope A Northrup; Brenda Porter; Gary R Cutter; Sarah E O'Kelley; Jessica Krefting; Scellig S Stone; Joseph R Madsen; Aria Fallah; Jeffrey P Blount; Howard L Weiner; E Martina Bebin Journal: Pediatr Neurol Date: 2020-04-14 Impact factor: 3.372
Authors: Romina Moavero; Katarzyna Kotulska; Lieven Lagae; Arianna Benvenuto; Leonardo Emberti Gialloreti; Bernhard Weschke; Kate Riney; Martha Feucht; Pavel Krsek; Rima Nabbout; Anna C Jansen; Konrad Wojdan; Julita Borkowska; Krzysztof Sadowski; Christoph Hertzberg; Monique M Van Schooneveld; Sharon Samueli; Alice Maulisovà; Eleonora Aronica; David J Kwiatkowski; Floor E Jansen; Sergiusz Jozwiak; Paolo Curatolo Journal: Ann Clin Transl Neurol Date: 2020-07-23 Impact factor: 4.511
Authors: Jessie De Ridder; Mario Lavanga; Birgit Verhelle; Jan Vervisch; Katrien Lemmens; Katarzyna Kotulska; Romina Moavero; Paolo Curatolo; Bernhard Weschke; Kate Riney; Martha Feucht; Pavel Krsek; Rima Nabbout; Anna C Jansen; Konrad Wojdan; Dorota Domanska-Pakieła; Magdalena Kaczorowska-Frontczak; Christoph Hertzberg; Cyrille H Ferrier; Sharon Samueli; Barbora Benova; Eleonora Aronica; David J Kwiatkowski; Floor E Jansen; Sergiusz Jóźwiak; Sabine Van Huffel; Lieven Lagae Journal: Front Neurol Date: 2020-10-16 Impact factor: 4.003