نتایج جستجو برای: ohtahara syndrome

تعداد نتایج: 621920  

Journal: :Clinical genetics 2012
H Saitsu M Kato M Shimono A Senju S Tanabe T Kimura K Nishiyama Y Yoneda Y Kondo Y Tsurusaki H Doi N Miyake K Hayasaka N Matsumoto

To the Editor : Ohtahara syndrome (OS) is characterized by early-onset of seizures, suppression-burst patterns on electroencephalogram (EEG), and severe psychomotor retardation (1–3). De novo mutations in the STXBP1 gene, including various point mutations and one complete deletion, have been found in about one-third of Japanese cases of cryptogenic OS (4–6). However, the clinical spectrum of ST...

Journal: :Neurology 2016
Hannah Stamberger Marina Nikanorova Marjolein H Willemsen Patrizia Accorsi Marco Angriman Hartmut Baier Ira Benkel-Herrenbrueck Valérie Benoit Mauro Budetta Almuth Caliebe Gaetano Cantalupo Giuseppe Capovilla Gianluca Casara Carolina Courage Marie Deprez Anne Destrée Robertino Dilena Corrie E Erasmus Madeleine Fannemel Roar Fjær Lucio Giordano Katherine L Helbig Henrike O Heyne Joerg Klepper Gerhard J Kluger Damien Lederer Monica Lodi Oliver Maier Andreas Merkenschlager Nina Michelberger Carlo Minetti Hiltrud Muhle Judith Phalin Keri Ramsey Antonino Romeo Jens Schallner Ina Schanze Marwan Shinawi Kristel Sleegers Katalin Sterbova Steffen Syrbe Monica Traverso Andreas Tzschach Peter Uldall Rudy Van Coster Helene Verhelst Maurizio Viri Susan Winter Markus Wolff Martin Zenker Leonardo Zoccante Peter De Jonghe Ingo Helbig Pasquale Striano Johannes R Lemke Rikke S Møller Sarah Weckhuysen

OBJECTIVE To give a comprehensive overview of the phenotypic and genetic spectrum of STXBP1 encephalopathy (STXBP1-E) by systematically reviewing newly diagnosed and previously reported patients. METHODS We recruited newly diagnosed patients with STXBP1 mutations through an international network of clinicians and geneticists. Furthermore, we performed a systematic literature search to review ...

2017
Anna‐Maria Katsarou Solomon L. Moshé Aristea S. Galanopoulou

GABAergic interneurons control the neural circuitry and network activity in the brain. The advances in genetics have identified genes that control the development, maturation and integration of GABAergic interneurons and implicated them in the pathogenesis of epileptic encephalopathies or neurodevelopmental disorders. For example, mutations of the Aristaless-Related homeobox X-linked gene (ARX)...

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