Purinergic control of hippocampal circuit hyperexcitability in Dravet syndrome

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Dravet in the dish: mechanisms of hyperexcitability.

Commentary Dravet syndrome, formerly known as Severe Myoclonic Epilepsy of Infancy (SMEI), is a devastating neurodevelopmental disorder of intractable epilepsy that begins in early infancy. Tragically, development of affected children is typically on track during the first year of life, whereas progressive developmental decline and prolonged seizures begin to emerge in the second year of life. ...

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Reduced dendritic arborization and hyperexcitability of pyramidal neurons in a Scn1b-based model of Dravet syndrome.

Epileptic encephalopathies, including Dravet syndrome, are severe treatment-resistant epilepsies with developmental regression. We examined a mouse model based on a human β1 sodium channel subunit (Scn1b) mutation. Homozygous mutant mice shared phenotypic features and pharmaco-sensitivity with Dravet syndrome. Patch-clamp analysis showed that mutant subicular and layer 2/3 pyramidal neurons had...

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Network Hyperexcitability in Hippocampal Slices

1 FROM Mecp2 MUTANT MICE REVEALED BY VOLTAGE2 SENSITIVE DYE IMAGING 3 4 Gaston Calfa, John J. Hablitz and Lucas Pozzo-Miller 5 6 Department of Neurobiology, Civitan International Research Center, 7 The University of Alabama at Birmingham, Birmingham, AL 35294, USA. 8 9 10 56 pages with 8 Figures, and 3 Supplemental Figures 11 12 Running title: Hippocampal hyperexcitability in Mecp2 mutant mice ...

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Dravet syndrome

"Dravet syndrome" (DS) previously named severe myoclonic epilepsy of infancy (SMEI), or epilepsy with polymorphic seizures, is a rare disorder characterized by an early, severe, generalized, epileptic encephalopathy.DS is characterized by febrile and afebrile seizures beginning in the 1st year of life followed by different types of seizures (either focal or generalized), which are typically res...

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Dravet Syndrome

Dravet syndrome (DS) is one of the refractory epileptic encephalopathies [3], one of the spectra of severe myoclonic Epilepsy of Infancy (SMEI) which occurs in otherwise healthy individuals [2]. Its incidence has been estimated to be 1 in 2000040000. It is more common in males than in females. Positive family history is encountered in one fourth of the cases. DS Usually started by clonic/tonic-...

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ژورنال

عنوان ژورنال: Epilepsia

سال: 2014

ISSN: 0013-9580

DOI: 10.1111/epi.12487