نتایج جستجو برای: early infantile epileptic encephalopathy

تعداد نتایج: 725871  

Journal: :Neurology. Genetics 2016
Karen L Oliver Vesna Lukic Saskia Freytag Ingrid E Scheffer Samuel F Berkovic Melanie Bahlo

OBJECTIVE To evaluate the performance of an in silico prioritization approach that was applied to 179 epileptic encephalopathy candidate genes in 2013 and to expand the application of this approach to the whole genome based on expression data from the Allen Human Brain Atlas. METHODS PubMed searches determined which of the 179 epileptic encephalopathy candidate genes had been validated. For v...

Background The X-linked cyclin-dependent kinase like 5 (CDKL5/STK9) gene has been shown to be responsible for a severe encephalopathy condition characterized by early onset of epilepsy and severe developmental delay. CDKL5 mutations have been shown to be more frequent among female patients. Results Here we report a 6- month male patient, second child of a healthy non consanguineous in the Irani...

Journal: :Annals of neurology 2014
Ali Torkamani Kevin Bersell Benjamin S Jorge Robert L Bjork Jennifer R Friedman Cinnamon S Bloss Julie Cohen Siddharth Gupta Sakkubai Naidu Carlos G Vanoye Alfred L George Jennifer A Kearney

OBJECTIVE Numerous studies have demonstrated increased load of de novo copy number variants or single nucleotide variants in individuals with neurodevelopmental disorders, including epileptic encephalopathies, intellectual disability, and autism. METHODS We searched for de novo mutations in a family quartet with a sporadic case of epileptic encephalopathy with no known etiology to determine t...

Journal: :Neurology 2017
Toni S Pearson Ingo Helbig

GNAO1 encephalopathy comprises a spectrum of neurologic phenotypes that result from de novo heterozygous mutations in GNAO1, a gene coding for the subunit of a G protein that is highly expressed in the CNS and is involved in second messenger signaling. De novo heterozygous mutations in the gene were first described in patients with a severe, infantile-onset epileptic encephalopathy known as Oht...

Journal: :Pediatric Neurology Briefs 2007

Journal: :Epilepsia 2015
Saadet Mercimek-Mahmutoglu Jaina Patel Dawn Cordeiro Stacy Hewson David Callen Elizabeth J Donner Cecil D Hahn Peter Kannu Jeff Kobayashi Berge A Minassian Mahendranath Moharir Komudi Siriwardena Shelly K Weiss Rosanna Weksberg O Carter Snead

OBJECTIVE Epilepsy is a common neurologic disorder of childhood. To determine the genetic diagnostic yield in epileptic encephalopathy, we performed a retrospective cohort study in a single epilepsy genetics clinic. METHODS We included all patients with intractable epilepsy, global developmental delay, and cognitive dysfunction seen between January 2012 and June 2014 in the Epilepsy Genetics ...

2014
Adeline Ngoh Amy McTague Ingrid M Wentzensen Esther Meyer Carolyn Applegate Eric H Kossoff Denise A Batista Tao Wang Manju A Kurian

Homozygous deletions of chromosome 20p12.3, disrupting the promoter region and first three coding exons of the phospholipase C β1 gene (PLCB1), have previously been described in two reports of early infantile epileptic encephalopathy (EIEE). Both children were born to consanguineous parents, one presented with infantile spasms, the other with migrating partial seizures of infancy. We describe a...

Journal: :Seizure 2016
Esra Türe Tülay Kamaşak Merve Cora Sevim Şahin Elif Acar Arslan Neşe Kaklıkaya Ali Cansu

PURPOSE Infantile spasm is an age-dependent epileptic syndrome seen in infancy or early childhood. Although studies have investigated the epilepsy-cytokine relationship, there has been insufficient research into the relation between cytokines and infantile spasm. The purpose of this study was to examine the role of cytokines in the pathogenesis of infantile spasm by investigating cytokine level...

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