TY - JOUR
T1 - Evolution of electroencephalogram in infants with tuberous sclerosis complex and neurodevelopmental outcome
T2 - a prospective cohort study
AU - the EPISTOP consortium
AU - De Ridder, Jessie
AU - Kotulska, Katarzyna
AU - Curatolo, Paolo
AU - Jansen, Anna C.
AU - Aronica, Eleonora
AU - Kwiatkowski, David J.
AU - Jansen, Floor E.
AU - Jóźwiak, Sergiusz
AU - Lagae, Lieven
AU - Anink, J.
AU - Benova, B.
AU - Benvenuto, A.
AU - Blazejczyk, M.
AU - Bongaarts, A.
AU - Borkowska, J.
AU - Breuillard, D.
AU - Chmielewski, D.
AU - Dabrowska, M.
AU - Domańska-Pakieła, D.
AU - Emberti Gialloreti, L.
AU - Ferrier, C. H.
AU - Feucht, M.
AU - Giannikou, K.
AU - Głowacka-Walas, J.
AU - Hamieh, L.
AU - Haręza, A.
AU - Hertzberg, C.
AU - Hulshof, H.
AU - Iyer, A.
AU - Janssen, B.
AU - Jaworski, J.
AU - Kaczorowska-Frontczak, M.
AU - Krsek, P.
AU - Lehmann, K.
AU - Lemmens, K.
AU - Leusman, A.
AU - Maćkowiak, N.
AU - Mills, J. D.
AU - Moavero, R.
AU - Muehlebner, A.
AU - Nabbout, R.
AU - Riney, K.
AU - Sadowski, K.
AU - Samueli, S.
AU - Scheldeman, C.
AU - Scholl, T.
AU - Schooneveld, M.
AU - Sciuto, A.
AU - Sijko, K.
AU - Slowinska, M.
AU - Tempes, A.
AU - Urbańska, M.
AU - van Scheppingen, J.
AU - Verhelle, B.
AU - Vervisch, J.
AU - Weschke, B.
AU - Wojdan, K.
N1 - Funding Information:
The members of the EPISTOP consortium are as follows: J Anink, B Benova, A Benvenuto, M Blazejczyk, A Bongaarts, J Borkowska, D Breuillard, D Chmielewski, M Dabrowska, D Domańska‐Pakieła, L Emberti Gialloreti, CH Ferrier, M Feucht, K Giannikou, J Głowacka‐Walas, L Hamieh, A Haręza, C Hertzberg, H Hulshof, A Iyer, B Janssen, J Jaworski, M Kaczorowska‐Frontczak, P Krsek, K Lehmann, K Lemmens, A Leusman, N Maćkowiak, JD Mills, R Moavero, A Muehlebner, R Nabbout, K Riney, K Sadowski, S Samueli, C Scheldeman, T Scholl, M Schooneveld, A Sciuto, K Sijko, M Slowinska, A Tempes, M Urbańska, J van Scheppingen, B Verhelle, J Vervisch, B Weschke, K Wojdan. This study was funded by the 7th Framework Programme of the European Commission within the Large‐Scale Integrating Project EPISTOP (grant no. 602391). Sergiusz Jóźwiak and Katarzyna Kotulska were partly financed by the EPIMARKER grant of the Polish National Center for Research and Development (no. STRATEGMED3/306306/4/2016). We thank all the patients who participated in the EPISTOP trial. We also thank all individuals and institutions that contributed to this study.
Publisher Copyright:
© 2021 Mac Keith Press.
PY - 2022/4
Y1 - 2022/4
N2 - Aim: To describe the evolution of electroencephalogram (EEG) characteristics in infants with tuberous sclerosis complex (TSC) and the relationship with neurodevelopmental outcome at 24 months. Method: Eighty-three infants were enrolled in the EPISTOP trial and underwent serial EEG follow-up until the age of 24 months (males n=45, females n=37, median age at enrolment 28d, interquartile range 14–54d). Maturation of the EEG background and epileptiform discharges were compared between the TSC1 and TSC2 variants and between preventive and conventional groups respectively. Results: Children with TSC2 more frequently had a slower posterior dominant rhythm (PDR) at 24 months (51% vs 11%, p=0.002), a higher number of epileptiform foci (median=8 vs 4, p=0.003), and a lower fraction of EEGs without epileptiform discharges (18% vs 61%, p=0.001) at follow-up. A slower PDR at 24 months was significantly associated with lower cognitive (median=70 vs 80, p=0.028) and motor developmental quotients (median=70 vs 79, p=0.008). A higher fraction of EEGs without epileptiform discharges was associated with a lower probability of autism spectrum disorder symptoms (odds ratio=0.092, 95% confidence interval=0.009–0.912, p=0.042) and higher cognitive (p=0.004), language (p=0.002), and motor (p=0.001) developmental quotients at 24 months. Interpretation: TSC2 is associated with more abnormal EEG characteristics compared to TSC1, which are predictive for neurodevelopmental outcome.
AB - Aim: To describe the evolution of electroencephalogram (EEG) characteristics in infants with tuberous sclerosis complex (TSC) and the relationship with neurodevelopmental outcome at 24 months. Method: Eighty-three infants were enrolled in the EPISTOP trial and underwent serial EEG follow-up until the age of 24 months (males n=45, females n=37, median age at enrolment 28d, interquartile range 14–54d). Maturation of the EEG background and epileptiform discharges were compared between the TSC1 and TSC2 variants and between preventive and conventional groups respectively. Results: Children with TSC2 more frequently had a slower posterior dominant rhythm (PDR) at 24 months (51% vs 11%, p=0.002), a higher number of epileptiform foci (median=8 vs 4, p=0.003), and a lower fraction of EEGs without epileptiform discharges (18% vs 61%, p=0.001) at follow-up. A slower PDR at 24 months was significantly associated with lower cognitive (median=70 vs 80, p=0.028) and motor developmental quotients (median=70 vs 79, p=0.008). A higher fraction of EEGs without epileptiform discharges was associated with a lower probability of autism spectrum disorder symptoms (odds ratio=0.092, 95% confidence interval=0.009–0.912, p=0.042) and higher cognitive (p=0.004), language (p=0.002), and motor (p=0.001) developmental quotients at 24 months. Interpretation: TSC2 is associated with more abnormal EEG characteristics compared to TSC1, which are predictive for neurodevelopmental outcome.
UR - http://www.scopus.com/inward/record.url?scp=85116455796&partnerID=8YFLogxK
U2 - 10.1111/dmcn.15073
DO - 10.1111/dmcn.15073
M3 - Article
C2 - 34601720
AN - SCOPUS:85116455796
SN - 0012-1622
VL - 64
SP - 495
EP - 501
JO - Developmental Medicine and Child Neurology
JF - Developmental Medicine and Child Neurology
IS - 4
ER -