Metabolic modulation of synaptic failure and thalamocortical hypersynchronization with preserved consciousness in Glut1 deficiency.
Animals
Blood Glucose
Carbohydrate Metabolism, Inborn Errors
Carbon
/ metabolism
Consciousness
Deoxyglucose
Electroencephalography
Glucose Transport Proteins, Facilitative
/ metabolism
Glycogen
/ metabolism
Mice
Monosaccharide Transport Proteins
/ deficiency
Seizures
Thalamus
/ metabolism
alpha-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic Acid
Journal
Science translational medicine
ISSN: 1946-6242
Titre abrégé: Sci Transl Med
Pays: United States
ID NLM: 101505086
Informations de publication
Date de publication:
05 10 2022
05 10 2022
Historique:
entrez:
5
10
2022
pubmed:
6
10
2022
medline:
12
10
2022
Statut:
ppublish
Résumé
Individuals with glucose transporter type I deficiency (G1D) habitually experience nutrient-responsive epilepsy associated with decreased brain glucose. However, the mechanistic association between blood glucose concentration and brain excitability in the context of G1D remains to be elucidated. Electroencephalography (EEG) in G1D individuals revealed nutrition time-dependent seizure oscillations often associated with preserved volition despite electrographic generalization and uniform average oscillation duration and periodicity, suggesting increased facilitation of an underlying neural loop circuit. Nonlinear EEG ictal source localization analysis and simultaneous EEG/functional magnetic resonance imaging converged on the thalamus-sensorimotor cortex as one potential circuit, and
Identifiants
pubmed: 36197967
doi: 10.1126/scitranslmed.abn2956
pmc: PMC10276203
mid: NIHMS1904657
doi:
Substances chimiques
Blood Glucose
0
Glucose Transport Proteins, Facilitative
0
Monosaccharide Transport Proteins
0
Carbon
7440-44-0
alpha-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic Acid
77521-29-0
Glycogen
9005-79-2
Deoxyglucose
9G2MP84A8W
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
eabn2956Subventions
Organisme : NINDS NIH HHS
ID : R01 NS077015
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS094257
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS102588
Pays : United States
Organisme : NINDS NIH HHS
ID : R35 NS116824
Pays : United States
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