CDKL5 deficiency disorder: progressive brain atrophy may be part of the syndrome

The clinical phenotype of Cyclin-Dependent Kinase-Like 5 (CDKL5) deficiency disorder (CDD) has been delineated but neuroimaging features have not been systematically analyzed. We studied brain magnetic resonance imaging (MRI) scans in a cohort of CDD patients and reviewed age at seizure onset, seizure semiology, head circumference. Thirty-five brain MRI from 22 unrelated patients were included. The median age at study entry was 13.4 years. In 14/22 patients (85.7%), MRI in the first year of life was unremarkable in all but two. In 11/22, we performed MRI after 24 months of age (range 2.5-23 years). In 8 out of 11 (72.7%), MRI showed supratentorial atrophy and in six cerebellar atrophy. Quantitative analysis detected volumetric reduction of the whole brain (-17.7%, P-value = 0.014), including both white matter (-25.7%, P-value = 0.005) and cortical gray matter (-9.1%, P-value = 0.098), with a reduction of surface area (-18.0%, P-value = 0.032), mainly involving the temporal regions, correlated with the head circumference (& rho; = 0.79, P-value = 0.109). Both the qualitative structural assessment and the quantitative analysis detected brain volume reduction involving the gray and white matter. These neuroimaging findings may be related to either progressive changes due to CDD pathogenesis, or to the extreme severity of epilepsy, or both. Larger prospective studies are needed to clarify the bases for the structural changes we observed

Foscolo critico

Il volume, primo della collana open access dei "Quaderni di Gargnano", ospita i contributi presentati al XV Convegno internazionale di Letteratura italiana "Gennaro Barbarisi", tenutosi a Gargnano del Garda dal 24 al 26 settembre 2012. Il "Quaderno", dedicato al Foscolo critico, accoglie contributi di Giovanni Biancardi, Arnaldo Bruni, Andrea Campana, Massimo Castellozzi, Gustavo Costa, Alfredo Cottignoli, Christian Del Vento, Sandro Gentili, Franco Longoni, Ilaria Mangiavacchi, Donatella Martinelli, Giuseppe Natale, Enzo Neppi, Matteo Palumbo, Elena Parrini Cantini, Chiara Piola Caselli. \uc8 aperto da una Prefazione di Claudia Berra, Paolo Borsa e Giulia Ravera.This volume on "Foscolo critico" is the first volume of the "Quaderni di Gargnano", an open access book series which publishes the Proceedings of the "Gennaro Barbarisi" International Conferences on Italian Literature, held in Gargnano del Garda. It contains contributions by Giovanni Biancardi, Arnaldo Bruni, Andrea Campana, Massimo Castellozzi, Gustavo Costa, Alfredo Cottignoli, Christian Del Vento, Sandro Gentili, Franco Longoni, Ilaria Mangiavacchi, Donatella Martinelli, Giuseppe Natale, Enzo Neppi, Matteo Palumbo, Elena Parrini Cantini, Chiara Piola Caselli, preceded by a Preface by the Editor: Claudia Berra, Paolo Borsa, Giulia Ravera

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Coronella austriaca (Smooth Snake) and Zamenis longissimus (Aesculapian Snake). Defensive Behavior.

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