Quantitative 1 H MR spectroscopic imaging in early Rett syndrome
Abstract
Objective To determine cerebral regional concentrations of N-acetyl aspartate (NAA), total choline (Cho), and total creatine (Cr) in Rett syndrome (RS) using 1H magnetic resonance spectroscopic imaging (MRSI). Background The biochemical defect underlying RS is unknown. Because in vivo MRSI can detect important cerebral metabolites, MRSI has a potential to reveal impairment of regional cerebral metabolism in RS noninvasively. Methods High-resolution, multislice 1H MRSI was carried out in 17 girls with RS. The control group consisted of nine healthy children. Results In patients with RS, average Cho concentration was 12% higher (p Conclusion Metabolic impairment in RS involves both gray and white matter and particularly involves frontal and parietal lobes and the insular cortex. Loss of NAA most likely reflects reduced neuronal and dendritic tree size; increased Cho concentration may result from gliosis.
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Changes in neurometabolites associated with cell integrity and membrane turnover have been reported in RTT.
“Changes in brain carbohydrate metabolism [20] and neurometabolites associated with cell integrity and membrane turnover [21,22] have also been reported.”
From Rett syndrome: a neurological disorder with metabolic components (2018) · cites this paper as [22]
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- Authors
- Horská, A., Naidu, S., Herskovits, E. H., Wang, P. Y., Kaufmann, W. E., & Barker, P. B.
- Affiliations
- From the Department of Radiology (Drs. HorskáHerskovits, Wang, and Barker); the Departments of Pathology, Neurology, Pediatrics, and Psychiatry (Dr. Kaufmann), Johns Hopkins University, School of Medicine; and the Neurogenetics Unit (Dr. Naidu), Kennedy Krieger Institute; Baltimore, MD
- Kennedy Krieger Institute (US)
- Venue
- Neurology
- Published
- 2000-02-08
- Volume
- 54
- Issue
- 3
- Pages
- 715-715
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- OpenAlex
- W2168568714
- Cited by
- 38
- References
- 66
- Citations by year
- 2023: 1 · 2022: 2 · 2021: 1 · 2018: 3 · 2015: 1 · 2014: 1 · 2013: 2
- FWCI
- 2.53
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Neurology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (P01HD024448, 2 P30 HD24061-11, 2 P01 HD24448-11, P30HD024061)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Advanced MRI Techniques and Applications
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Fetal and Pediatric Neurological Disorders
- Keywords
- Rett syndrome, N-acetylaspartate, total choline, total creatine, brain metabolites, regional brain metabolism, Cho/NAA ratio, NAA/Cr ratio, insula, frontal lobe, parietal lobe, hippocampus, seizures, gliosis, neuronal loss, 1H magnetic resonance spectroscopic imaging
- MeSH terms
- Analysis of Variance, Brain, Child, Child, Preschool, Female, Humans, Magnetic Resonance Spectroscopy, Rett Syndrome
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