scholarly journals Subcortical band heterotopia

2020 ◽  
Author(s):  
Keyword(s):  
Subcortical Band Heterotopia ◽  
Band Heterotopia

scholarly journals Direct and Collateral Alterations of Functional Cortical Circuits in a Rat Model of Subcortical Band Heterotopia

2018 ◽  
Vol 29 (10) ◽  
pp. 4253-4262 ◽  
Author(s):  
Vanessa Plantier ◽  
Françoise Watrin ◽  
Emmanuelle Buhler ◽  
Fanny Sandrine Martineau ◽  
Surajit Sahu ◽  
...  
Keyword(s):  
Barrel Cortex ◽  
Intractable Epilepsy ◽  
Pyramidal Cells ◽  
Cortical Region ◽  
Cortical Networks ◽  
Causative Gene ◽  
Double Cortex Syndrome ◽  
Subcortical Band Heterotopia ◽  
Migration Disorder ◽  
Band Heterotopia

Abstract Subcortical band heterotopia (SBH), also known as double-cortex syndrome, is a neuronal migration disorder characterized by an accumulation of neurons in a heterotopic band below the normotopic cortex. The majority of patients with SBH have mild to moderate intellectual disability and intractable epilepsy. However, it is still not clear how cortical networks are organized in SBH patients and how this abnormal organization contributes to improper brain function. In this study, cortical networks were investigated in the barrel cortex in an animal model of SBH induced by in utero knockdown of Dcx, main causative gene of this condition in human patients. When the SBH was localized below the Barrel Field (BF), layer (L) four projection to correctly positioned L2/3 pyramidal cells was weakened due to lower connectivity. Conversely, when the SBH was below an adjacent cortical region, the excitatory L4 to L2/3 projection was stronger due to increased L4 neuron excitability, synaptic strength and excitation/inhibition ratio of L4 to L2/3 connection. We propose that these developmental alterations contribute to the spectrum of clinical dysfunctions reported in patients with SBH.

scholarly journals Characterization of the HeCo Mutant Mouse: A New Model of Subcortical Band Heterotopia Associated with Seizures and Behavioral Deficits

2008 ◽  
Vol 19 (3) ◽  
pp. 563-575 ◽  
Author(s):  
Alexandre Croquelois ◽  
Fabienne Giuliani ◽  
Christine Savary ◽  
Michel Kielar ◽  
Clotilde Amiot ◽  
...  
Keyword(s):  
Mutant Mouse ◽  
Behavioral Deficits ◽  
New Model ◽  
Subcortical Band Heterotopia ◽  
Band Heterotopia

Mosaic DCX deletion causes subcortical band heterotopia in males

Neurogenetics ◽  
2012 ◽  
Vol 13 (4) ◽  
pp. 367-373 ◽  
Author(s):  
Chloé Quélin ◽  
Yoann Saillour ◽  
Isabelle Souville ◽  
Karine Poirier ◽  
Marie Ange N’Guyen-Morel ◽  
...  
Keyword(s):  
Subcortical Band Heterotopia ◽  
Band Heterotopia

Mosaic mutations of the LIS1 gene cause subcortical band heterotopia

Neurology ◽  
2003 ◽  
Vol 61 (8) ◽  
pp. 1042-1046 ◽  
Author(s):  
F. Sicca ◽  
A. Kelemen ◽  
P. Genton ◽  
S. Das ◽  
D. Mei ◽  
...  
Keyword(s):  
Subcortical Band Heterotopia ◽  
Band Heterotopia

Neuroanatomical characterization of the cellular and axonal architecture of subcortical band heterotopia in the BXD29-Tlr4lps-2J/J mouse cortex

Neuroscience ◽  
2016 ◽  
Vol 337 ◽  
pp. 48-65 ◽  
Author(s):  
Raddy L. Ramos ◽  
Alyssa R. Toia ◽  
Daniel M. Pasternack ◽  
Timothy P. Dotzler ◽  
Joshua A. Cuoco ◽  
...  
Keyword(s):  
Mouse Cortex ◽  
Subcortical Band Heterotopia ◽  
Band Heterotopia

scholarly journals Spared cognitive and behavioral functions prior to epilepsy onset in a rat model of subcortical band heterotopia

2019 ◽  
Vol 1711 ◽  
pp. 146-155
Author(s):  
Fanny Sandrine Martineau ◽  
Lauriane Fournier ◽  
Emmanuelle Buhler ◽  
Françoise Watrin ◽  
Francesca Sargolini ◽  
...  
Keyword(s):  
Rat Model ◽  
Subcortical Band Heterotopia ◽  
Band Heterotopia

Males with epilepsy, complete subcortical band heterotopia, and somatic mosaicism for DCX

Neurology ◽  
2002 ◽  
Vol 58 (10) ◽  
pp. 1559-1562 ◽  
Author(s):  
N. P. Poolos ◽  
S. Das ◽  
G. D. Clark ◽  
D. Lardizabal ◽  
J. L. Noebels ◽  
...  
Keyword(s):  
Somatic Mosaicism ◽  
Subcortical Band Heterotopia ◽  
Band Heterotopia

scholarly journals Mutation analysis of the DCX gene and genotype/phenotype correlation in subcortical band heterotopia

2001 ◽  
Vol 9 (1) ◽  
pp. 5-12 ◽  
Author(s):  
Naomichi Matsumoto ◽  
RichardJ Leventer ◽  
JulieA Kuc ◽  
StephanieK Mewborn ◽  
Laura L Dudlicek ◽  
...  
Keyword(s):  
Mutation Analysis ◽  
Phenotype Correlation ◽  
Genotype Phenotype Correlation ◽  
Subcortical Band Heterotopia ◽  
Band Heterotopia
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