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1 Dravet inhibitory neurons showed deficits in sodium curr
2 Dravet syndrome (also called severe myoclonic epilepsy o
3 Dravet syndrome (DS) is a catastrophic developmental and
4 Dravet syndrome (DS) is a devastating early-onset refrac
5 Dravet syndrome (DS) is a form of epilepsy with a high i
6 Dravet syndrome (DS) is a neurodevelopmental disorder ch
7 Dravet syndrome (DS) is a neurodevelopmental disorder du
8 Dravet syndrome (DS) is a severe developmental epileptic
9 Dravet syndrome (DS) is a severe early-onset epilepsy as
10 Dravet syndrome (DS) is a severe form of epilepsy arisin
11 Dravet Syndrome (DS) is a severe neurodevelopmental diso
12 Dravet syndrome (DS) is an epileptic encephalopathy that
13 Dravet syndrome (DS) is an infantile-onset intractable e
14 Dravet syndrome (DS) is one of the most severe childhood
15 Dravet syndrome (DS), an intractable childhood epileptic
16 Dravet syndrome is a catastrophic childhood epilepsy wit
17 Dravet syndrome is a catastrophic pediatric epilepsy wit
18 Dravet syndrome is a rare, treatment-resistant developme
19 Dravet Syndrome is a severe childhood epileptic disorder
20 Dravet syndrome is a severe epileptic encephalopathy cau
21 Dravet syndrome is a severe infantile onset epileptic en
22 Dravet syndrome is an archetypal rare severe epilepsy, c
23 Dravet syndrome is an epilepsy syndrome of infantile ons
24 Dravet syndrome is an infant-onset epileptic encephalopa
25 Dravet syndrome is an intractable developmental and epil
26 Dravet Syndrome is an intractable form of childhood epil
27 Dravet syndrome is the prototype of SCN1A-mutation assoc
28 Dravet syndrome mice (Scn1a(+/-) ) demonstrate a marked
29 Dravet syndrome parvalbumin-positive interneuron sodium
30 Dravet syndrome, an epileptic encephalopathy affecting c
31 logical level rescued seizures also in adult Dravet syndrome mice (P90) after months of repetitive at
33 ural variation was found in 60% of the adult Dravet patients tested, including one post-mortem case w
34 lepsy syndromes are mild and remit with age, Dravet syndrome has a more severe clinical course with r
35 , such as autism spectrum disorder (ASD) and Dravet, Fragile X, Prader-Willi, Turner, and Williams sy
38 seizures associated with Lennox-Gastaut and Dravet syndromes but appears not yet to have been establ
39 epsy with febrile seizures plus (GEFS+), and Dravet syndrome (DS)/severe myoclonic epilepsy in infanc
40 zed epilepsy with febrile seizures plus, and Dravet syndrome or severe myoclonic epilepsy in infancy.
41 for treatment of Lennox-Gastaut syndrome and Dravet syndrome, two severe childhood-onset epilepsies,
43 ptic drug used to treat children affected by Dravet syndrome, possibly by inhibiting neuronal lactate
44 s in the brain sodium channel Na(V)1.1 cause Dravet syndrome (DS), a pharmacoresistant infantile-onse
45 voltage-gated sodium channel Na(V)1.1 causes Dravet's syndrome, a childhood neuropsychiatric disorder
46 1.1 truncation mutation (R1407X) that causes Dravet syndrome in humans, and examined their survival,
48 nimal models of the epileptic encephalopathy Dravet syndrome (DS), including the one employed in our
49 ice, a model of the epileptic encephalopathy Dravet syndrome, in which patients experience spontaneou
51 -function variants cause the severe epilepsy Dravet syndrome, as well as milder phenotypes associated
52 perature-induced seizures in male and female Dravet syndrome (Scn1a+/-) mice, a neurodevelopmental di
54 benzodiazepines are a first-line therapy for Dravet syndrome, they are limited by their ability to on
57 ised genomic background to generate the full Dravet syndrome phenotype, whilst genomic resilience may
58 In the safety group, 33 (20%) patients had Dravet syndrome and 31 (19%) patients had Lennox-Gastaut
59 panel of drugs in a zebrafish model of human Dravet syndrome, we show that even drugs with related me
62 luding those associated with astrogliosis in Dravet syndrome mice, that, accordingly, were rescued by
65 u reduction may be of therapeutic benefit in Dravet syndrome and other intractable genetic epilepsies
66 fficacy of a novel sodium channel blocker in Dravet syndrome and suggest a potential mechanism involv
69 to cortical pyramidal neurons was reduced in Dravet syndrome mice, suggesting decreased GABA release
72 state during temperature-induced seizures in Dravet syndrome.SIGNIFICANCE STATEMENT Epilepsy is a com
75 tal and epileptic encephalopathies including Dravet syndrome with common SCN1A variants being risk fa
77 for multiple epilepsy phenotypes, including Dravet syndrome, febrile seizures (FS) and genetic epile
79 dings, we treated five medically intractable Dravet syndrome patients with a clinically-approved sero
81 ne particularly devastating channelopathy is Dravet syndrome (DS), a severe childhood epilepsy typica
84 B p.R125C is an autosomal recessive cause of Dravet syndrome through functional gene inactivation.
88 se model that recapitulates many features of Dravet syndrome, including spontaneous seizures, prematu
90 s in a well-validated mouse genetic model of Dravet syndrome (DS), a severe childhood epilepsy disord
91 ated to be seizure resistant in the model of Dravet syndrome (Scn1a(+/-) ), and in which the alpha(2)
92 calcium imaging in an experimental model of Dravet syndrome (Scn1a+/- mice)-a severe neurodevelopmen
93 hibitory neural activity in a mouse model of Dravet syndrome disinhibited RTN chemoreceptors and dest
96 nd autism-like behaviors in a mouse model of Dravet syndrome, a severe epileptic encephalopathy of ea
97 ence of SGE-516 activity in a mouse model of Dravet syndrome, and supports further investigation of n
98 ever, in contrast to the Scn1a(+/-) model of Dravet syndrome, we found no measurable differences in s
102 in brain slices from genetic mouse models of Dravet syndrome (DS) reveal reduced sodium current and e
103 ive seizures in patients and mouse models of Dravet syndrome (DS), a developmental and epileptic ence
105 Tau ablation prevented the high mortality of Dravet mice and reduced the frequency of spontaneous and
109 ns had clinical features resembling those of Dravet syndrome with progression toward atypical absence
110 n with haploinsufficiency that is typical of Dravet syndrome and could readily explain the more sever
111 evere myoclonic epilepsy of infancy (SMEI or Dravet's Syndrome), which includes severe, intractable e
120 myoclonic-astatic epilepsy (Doose syndrome), Dravet syndrome, and status epilepticus (including FIRES
121 y syndromes such as Lennox-Gastaut syndrome, Dravet syndrome and infantile spasms with intellectual d
122 tment of infantile epileptic spasm syndrome, Dravet syndrome, and Lennox-Gastaut syndrome, with a foc
124 .1 mutation and supports the hypothesis that Dravet Syndrome arises from defective inhibitory neurons
127 igned 120 children and young adults with the Dravet syndrome and drug-resistant seizures to receive e
129 sociated with gain of function, different to Dravet syndrome variants (odds ratio = 17.8; confidence
138 ounteract brain dysfunctions associated with Dravet syndrome and that overall cerebral TAU reduction
139 -mortem material from three adult cases with Dravet syndrome, in comparison with controls and a range
141 cted data on a UK cohort of individuals with Dravet syndrome during a 5-year study period and analyse
145 A gene have been identified in patients with Dravet's syndrome (severe myoclonic epilepsy of infancy)
146 p.S1328P) identified in a pair of twins with Dravet Syndrome and generated iPSC-derived neurons from