نتایج جستجو برای: early infantile epileptic encephalopathy

تعداد نتایج: 725871  

Journal: :Journal of child neurology 2011
Carl E Stafstrom Barry G W Arnason Tallie Z Baram Anna Catania Miguel A Cortez Tracy A Glauser Michael R Pranzatelli Raili Riikonen Michael A Rogawski Shlomo Shinnar John W Swann

Infantile spasms is an epileptic encephalopathy of early infancy with specific clinical and electroencephalographic (EEG) features, limited treatment options, and a poor prognosis. Efforts to develop improved treatment options have been hindered by the lack of experimental models in which to test prospective therapies. The neuropeptide adrenocorticotropic hormone (ACTH) is effective in many cas...

Journal: :Arquivos De Neuro-psiquiatria 2023

Background: West syndrome (WS) is an epileptic encephalopathy characterized by spasms, neurodevelopment delay, and hypsarrhythmia on electroencephalography. Infantile spasms have incidence of about 0.43 per 1000 livebirths occur between 3 12 months age. The drug's choice the adrenocorticotrophic hormone (ACTH), with varied response rates limited efficacy data, relapses, evolution to other kinds...

Journal: :Neuropediatrics 2014
Annette Hackenberg Alessandra Baumer Heinrich Sticht Bernhard Schmitt Judith Kroell-Seger David Wille Pascal Joset Sorina Papuc Anita Rauch Barbara Plecko

Mutations of the SCN2A gene have originally been described in association with benign familial neonatal-infantile seizures (BFNIS). Recently, single patients with more severe phenotypes and persisting epileptic encephalopathies have been recognized. We report the case of a girl with severe infantile onset epileptic encephalopathy and a de novo missense mutation in the SCN2A gene (c.4025T > C/ =...

2016
Janet Malcolmson Robert Kleyner David Tegay Whit Adams Kenneth Ward Justine Coppinger Lesa Nelson Miriam H. Meisler Kai Wang Reid Robison Gholson J. Lyon

The SCN8A gene encodes the sodium voltage-gated channel alpha subunit 8. Mutations in this gene have been associated with early infantile epileptic encephalopathy type 13. With the use of whole-exome sequencing, a de novo missense mutation in SCN8A was identified in a 4-yr-old female who initially exhibited symptoms of epilepsy at the age of 5 mo that progressed to a severe condition with very ...

Journal: :Journal of pediatric epilepsy 2014
Mahsa Parviz Kara Vogel K Michael Gibson Phillip L Pearl

Clinical disorders known to affect inherited gamma-amino butyric acid (GABA) metabolism are autosomal recessively inherited succinic semialdehyde dehydrogenase and GABA-transaminase deficiency. The clinical presentation of succinic semialdehyde dehydrogenase deficiency includes intellectual disability, ataxia, obsessive-compulsive disorder and epilepsy with a nonprogressive course in typical ca...

2015
M. G. Aartsen M. Ackermann J. Adams J. A. Aguilar M. Ahlers M. Ahrens D. Altmann T. Anderson C. Arguelles T. C. Arlen J. Auffenberg X. Bai S. W. Barwick V. Baum R. Bay J. J. Beatty J. Becker Tjus K.-H. Becker S. BenZvi P. Berghaus D. Berley E. Bernardini A. Bernhard D. Z. Besson G. Binder D. Bindig M. Bissok E. Blaufuss J. Blumenthal D. J. Boersma C. Bohm F. Bos D. Bose S. Böser O. Botner L. Brayeur H.-P. Bretz A. M. Brown N. Buzinsky J. Casey M. Casier E. Cheung D. Chirkin A. Christov B. Christy K. Clark L. Classen F. Clevermann S. Coenders D. F. Cowen J. C. Davis M. Day J. P. A. M. de André C. De Clercq S. De Ridder P. Desiati K. D. de Vries M. de With T. DeYoung J. C. Díaz- Vélez M. Dunkman R. Eagan B. Eberhardt T. Ehrhardt B. Eichmann J. Eisch S. Euler P. A. Evenson O. Fadiran A. R. Fazely A. Fedynitch S. Flis K. Frantzen T. Fuchs T. K. Gaisser R. Gaior J. Gallagher L. Gerhardt D. Gier L. Gladstone T. Glüsenkamp A. Goldschmidt G. Golup J. G. Gonzalez J. A. Goodman D. Góra D. Grant P. Gretskov J. C. Groh C. Ha C. Haack A. Haj Ismail P. Hallen A. Hallgren F. Halzen K. Hanson D. Hebecker D. Heinen K. Helbing R. Hellauer D. Hellwig S. Hickford G. C. Hill K. D. Hoffman A. Homeier K. Hoshina F. Huang W. Huelsnitz P. O. Hulth K. Hultqvist A. Ishihara J. Jacobsen G. S. Japaridze K. Jero O. Jlelati M. Jurkovic B. Kaminsky A. Kappes T. Karg M. Kauer A. Keivani J. L. Kelley A. Kheirandish J. Kiryluk J. Kläs S. R. Klein G. Kohnen H. Kolanoski A. Koob L. Köpke C. Kopper S. Kopper D. J. Koskinen A. Kriesten K. Krings G. Kroll M. Kroll J. Kunnen N. Kurahashi T. Kuwabara J. L. Lanfranchi D. T. Larsen M. J. Larson G. Maggi R. Maruyama K. Mase H. S. Matis R. Maunu F. McNally K. Meagher M. Medici E. Middlemas N. Milke J. Miller L. Mohrmann U. Naumann H. Niederhausen S. C. Nowicki D. R. Nygren S. Odrowski A. Olivas A. Omairat A. O’Murchadha T. Palczewski D. Pieloth E. Pinat J. Posselt P. B. Price G. T. Przybylski J. Pütz M. Quinnan L. Rädel M. Rameez K. Rawlins P. Redl I. Rees R. Reimann E. Resconi W. Rhode M. Richman B. Riedel S. Robertson J. P. Rodrigues M. Rongen

We present constraints derived from a search of four years of IceCube data for a prompt neutrino flux from gammaray bursts (GRBs). A single low-significance neutrino, compatible with the atmospheric neutrino background, was found in coincidence with one of the 506 observed bursts. Although GRBs have been proposed as candidate sources for ultra-high-energy cosmic rays, our limits on the neutrino...

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