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3D reconstructions of local synaptic connectivity in hippocampal CA1 pyramidal neurons obtained by volume electron microscopy. Dendritic segments (gray) are shown either alone or with their innervating axons in a wild-type mouse (left) and in a Cyfip2+/R87C knock-in mouse model of West syndrome (right). Yellow puncta indicate postsynaptic densities. Compared with wild-type neurons, knock-in neurons exhibit markedly reduced dendritic spines, with excitatory synapses forming directly on dendritic shafts and showing dense clustering of presynaptic axon terminals. Scale bar, 1 µm. Image credit: Kea Joo Lee and Gyu Hyun Kim.

3D reconstructions of local synaptic connectivity in hippocampal CA1 pyramidal neurons obtained by volume electron microscopy. Dendritic segments (gray) are shown either alone or with their innervating axons in a wild-type mouse (left) and in a Cyfip2+/R87C knock-in mouse model of West syndrome (right). Yellow puncta indicate postsynaptic densities. Compared with wild-type neurons, knock-in neurons exhibit markedly reduced dendritic spines, with excitatory synapses forming directly on dendritic shafts and showing dense clustering of presynaptic axon terminals. Scale bar, 1 µm. Image credit: Kea Joo Lee and Gyu Hyun Kim.

#WestSyndrome is a severe neurodevelopmental disorder. This study provides a longitudinal picture of glial activation, synaptic remodeling & #LipidMetabolism in a mouse model of West syndrome, uncovering cellular & molecular mechanisms linked to #seizure evolution @plosbiology.org 🧪 plos.io/48tgUkv

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3D reconstructions of local synaptic connectivity in hippocampal CA1 pyramidal neurons obtained by volume electron microscopy. Dendritic segments (gray) are shown either alone or with their innervating axons in a wild-type mouse (left) and in a Cyfip2+/R87C knock-in mouse model of West syndrome (right). Yellow puncta indicate postsynaptic densities. Compared with wild-type neurons, knock-in neurons exhibit markedly reduced dendritic spines, with excitatory synapses forming directly on dendritic shafts and showing dense clustering of presynaptic axon terminals. Scale bar, 1 µm. Image credit: Kea Joo Lee and Gyu Hyun Kim.

3D reconstructions of local synaptic connectivity in hippocampal CA1 pyramidal neurons obtained by volume electron microscopy. Dendritic segments (gray) are shown either alone or with their innervating axons in a wild-type mouse (left) and in a Cyfip2+/R87C knock-in mouse model of West syndrome (right). Yellow puncta indicate postsynaptic densities. Compared with wild-type neurons, knock-in neurons exhibit markedly reduced dendritic spines, with excitatory synapses forming directly on dendritic shafts and showing dense clustering of presynaptic axon terminals. Scale bar, 1 µm. Image credit: Kea Joo Lee and Gyu Hyun Kim.

#WestSyndrome is a severe neurodevelopmental disorder. This study provides a longitudinal picture of glial activation, synaptic remodeling & #LipidMetabolism in a mouse model of West syndrome, uncovering cellular & molecular mechanisms linked to #seizure evolution @plosbiology.org 🧪 plos.io/48tgUkv

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3D reconstructions of local synaptic connectivity in hippocampal CA1 pyramidal neurons obtained by volume electron microscopy. Dendritic segments (gray) are shown either alone or with their innervating axons in a wild-type mouse (left) and in a Cyfip2+/R87C knock-in mouse model of West syndrome (right). Yellow puncta indicate postsynaptic densities. Compared with wild-type neurons, knock-in neurons exhibit markedly reduced dendritic spines, with excitatory synapses forming directly on dendritic shafts and showing dense clustering of presynaptic axon terminals. Scale bar, 1 µm. Image credit: Kea Joo Lee and Gyu Hyun Kim.

3D reconstructions of local synaptic connectivity in hippocampal CA1 pyramidal neurons obtained by volume electron microscopy. Dendritic segments (gray) are shown either alone or with their innervating axons in a wild-type mouse (left) and in a Cyfip2+/R87C knock-in mouse model of West syndrome (right). Yellow puncta indicate postsynaptic densities. Compared with wild-type neurons, knock-in neurons exhibit markedly reduced dendritic spines, with excitatory synapses forming directly on dendritic shafts and showing dense clustering of presynaptic axon terminals. Scale bar, 1 µm. Image credit: Kea Joo Lee and Gyu Hyun Kim.

#WestSyndrome is a severe neurodevelopmental disorder. This study provides a longitudinal picture of glial activation, synaptic remodeling & #LipidMetabolism in a mouse model of West syndrome, uncovering cellular & molecular mechanisms linked to #seizure evolution @plosbiology.org 🧪 plos.io/48tgUkv

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Key point: We hypothesized that the time course of the BASED score after ES remission is associated with ES relapse.
doi.org/10.1111/epi....

#epilepsy #ilae #antiseizuremedication #electroencephalography #hypsarrhythmia #interraterreliability #Westsyndrome

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Key point: Thalamic electroencephalography (EEG) showed slow-wave complex with or without low-voltage fast activity during epileptic spasms.
onlinelibrary.wiley.com/doi/full/10....

#epilepsy #ILAE #corticothalamicnetwork #infantileepilepticspasmssyndrome #thalamus #westsyndrome

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Key point: Racial disparities: Non-Hispanic Black children with IESS had significantly lower odds of undergoing genetic sequencing.
doi.org/10.1002/epi4...

#epilepsy #ILAE #geneticsequencing #healthequity #inequities #infantilespasms #westsyndrome

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