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The effect of chronic neuroglycopenia on resting state networks in GLUT1 syndrome across the lifespan.
- Source :
-
Human brain mapping [Hum Brain Mapp] 2020 Feb 01; Vol. 41 (2), pp. 453-466. Date of Electronic Publication: 2019 Nov 11. - Publication Year :
- 2020
-
Abstract
- Glucose transporter type I deficiency syndrome (GLUT1DS) is an encephalopathic disorder due to a chronic insufficient transport of glucose into the brain. PET studies in GLUT1DS documented a widespread cortico-thalamic hypometabolism and a signal increase in the basal ganglia, regardless of age and clinical phenotype. Herein, we captured the pattern of functional connectivity of distinct striatal, cortical, and cerebellar regions in GLUT1DS (10 children, eight adults) and in healthy controls (HC, 19 children, 17 adults) during rest. Additionally, we explored for regional connectivity differences in GLUT1 children versus adults and according to the clinical presentation. Compared to HC, GLUT1DS exhibited increase connectivity within the basal ganglia circuitries and between the striatal regions with the frontal cortex and cerebellum. The excessive connectivity was predominant in patients with movement disorders and in children compared to adults, suggesting a correlation with the clinical phenotype and age at fMRI study. Our findings highlight the primary role of the striatum in the GLUT1DS pathophysiology and confirm the dependency of symptoms to the patients' chronological age. Despite the reduced chronic glucose uptake, GLUT1DS exhibit increased connectivity changes in regions highly sensible to glycopenia. Our results may portrait the effect of neuroprotective brain strategy to overcome the chronic poor energy supply during vulnerable ages.<br /> (© 2019 The Authors. Human Brain Mapping published by Wiley Periodicals, Inc.)
- Subjects :
- Adolescent
Adult
Child
Chronic Disease
Epilepsy diagnostic imaging
Epilepsy etiology
Epilepsy metabolism
Epilepsy physiopathology
Female
Humans
Magnetic Resonance Imaging
Male
Movement Disorders diagnostic imaging
Movement Disorders etiology
Movement Disorders metabolism
Movement Disorders physiopathology
Young Adult
Basal Ganglia diagnostic imaging
Basal Ganglia metabolism
Basal Ganglia physiopathology
Brain Diseases, Metabolic, Inborn diagnostic imaging
Brain Diseases, Metabolic, Inborn genetics
Brain Diseases, Metabolic, Inborn metabolism
Brain Diseases, Metabolic, Inborn physiopathology
Cerebellum diagnostic imaging
Cerebellum metabolism
Cerebellum physiopathology
Glucose Transporter Type 1 deficiency
Human Development
Nerve Net diagnostic imaging
Nerve Net metabolism
Nerve Net physiopathology
Neuroprotection
Prefrontal Cortex diagnostic imaging
Prefrontal Cortex metabolism
Prefrontal Cortex physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 1097-0193
- Volume :
- 41
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Human brain mapping
- Publication Type :
- Academic Journal
- Accession number :
- 31710770
- Full Text :
- https://doi.org/10.1002/hbm.24815