نتایج جستجو برای: scn1a mutations

تعداد نتایج: 173129  

Journal: :Brain : a journal of neurology 2012
Charlotte Dravet

In this issue of Brain, Brunklaus et al. report a study of 241 patients with Dravet syndrome carrying a SCN1A mutation, with the aim of identifying predictors of developmental outcome and determining specific clinical and demographic characteristics. The electroclinical features of a large cohort were collected and analysed prior to genetic testing. A total of 355 patients were diagnosed with D...

2013
Dalia Kasperavičiūtė Claudia B. Catarino Mar Matarin Costin Leu Jan Novy Anna Tostevin Bárbara Leal Ellen V. S. Hessel Kerstin Hallmann Michael S. Hildebrand Hans-Henrik M. Dahl Mina Ryten Daniah Trabzuni Adaikalavan Ramasamy Saud Alhusaini Colin P. Doherty Thomas Dorn Jörg Hansen Günter Krämer Bernhard J. Steinhoff Dominik Zumsteg Susan Duncan Reetta K. Kälviäinen Kai J. Eriksson Anne-Mari Kantanen Massimo Pandolfo Ursula Gruber-Sedlmayr Kurt Schlachter Eva M. Reinthaler Elisabeth Stogmann Fritz Zimprich Emilie Théâtre Colin Smith Terence J. O’Brien K. Meng Tan Slave Petrovski Angela Robbiano Roberta Paravidino Federico Zara Pasquale Striano Michael R. Sperling Russell J. Buono Hakon Hakonarson João Chaves Paulo P. Costa Berta M. Silva António M. da Silva Pierre N. E. de Graan Bobby P. C. Koeleman Albert Becker Susanne Schoch Marec von Lehe Philipp S. Reif Felix Rosenow Felicitas Becker Yvonne Weber Holger Lerche Karl Rössler Michael Buchfelder Hajo M. Hamer Katja Kobow Roland Coras Ingmar Blumcke Ingrid E. Scheffer Samuel F. Berkovic Michael E. Weale Norman Delanty Chantal Depondt Gianpiero L. Cavalleri Wolfram S. Kunz Sanjay M. Sisodiya

Epilepsy comprises several syndromes, amongst the most common being mesial temporal lobe epilepsy with hippocampal sclerosis. Seizures in mesial temporal lobe epilepsy with hippocampal sclerosis are typically drug-resistant, and mesial temporal lobe epilepsy with hippocampal sclerosis is frequently associated with important co-morbidities, mandating the search for better understanding and treat...

Journal: :Cephalalgia : an international journal of headache 2008
E Cuenca-León R Corominas N Fernàndez-Castillo V Volpini M Del Toro M Roig A Macaya B Cormand

Familial hemiplegic migraine (FHM) is a rare type of migraine with aura. Mutations in three genes have been described in FHM patients: CACNA1A (FHM1), ATP1A2 (FHM2) and SCN1A (FHM3). We screened 27 Spanish patients with hemiplegic migraine (HM), basilar-type migraine or childhood periodic syndromes (CPS) for mutations in these three genes. Two novel CACNA1A variants, p.Val581Met and p.Tyr1245Cy...

Journal: :Epilepsy currents 2014
Ravi Chopra Lori L Isom

Dravet syndrome (also known as Severe Myoclonic Epilepsy of Infancy) is a rare genetic epilepsy syndrome commonly associated with loss-of-function mutations in SCN1A, the gene encoding the α subunit of the voltage-gated sodium channel NaV1.1, resulting in haploinsufficiency. Like other voltage-gated sodium channels, NaV1.1 function contributes to the rising phase of the neuronal action potentia...

2002
Alexis Hernández

We study compact non-supersymmetric Z N orbifolds in various dimensions. We compute the spectrum of several tachyonic type II and heterotic examples and partially classify tachyon-free heterotic models. We also discuss the relation to compactification on K3 and Calabi-Yau manifolds.

Journal: :Arquivos de neuro-psiquiatria 2006
Luciana R Lopes Mario Fernando Prieto Peres Kaate R J Vanmolkot Patrícia R Tobo Eliova Zukerman Rune R Frants Arn M J M van den Maagdenberg Carlos Alberto Moreira-Filho

Familial hemiplegic migraine (FHM) is a rare autosomal dominant form of migraine with aura. This disease has been associated with missense mutations in the CACNA1A and ATP1A2 genes. The aim of this study was to identify whether CACNA1A and ATP1A2 are or not related to Brazilian FHM. Here we screened four Brazilian FHM families (total of 26 individuals--13 affected and 13 asymptomatic or normal)...

Journal: :Epilepsy research 2014
François Le Gal Sébastien Lebon Gian Paolo Ramelli Alexandre N Datta Danielle Mercati Oliver Maier Christophe Combescure Maria Isabel Rodriguez Margitta Seeck Eliane Roulet Christian M Korff

PURPOSE To identify clinical risk factors for Dravet syndrome (DS) in a population of children with status epilepticus (SE). MATERIAL AND METHODS Children aged between 1 month and 16 years with at least one episode of SE were referred from 6 pediatric neurology centers in Switzerland. SE was defined as a clinical seizure lasting for more than 30min without recovery of normal consciousness. Th...

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