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Titolo:
Quantitative H-1 MR spectroscopic imaging in early Rett syndrome
Autore:
Horska, A; Naidu, S; Herskovits, EH; Wang, PY; Kaufmann, WE; Barker, PB;
Indirizzi:
Johns Hopkins Univ, Sch Med, Dept Radiol, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 Radiol, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Pathol, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 Pathol, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Neurol, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 Neurol, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Pediat, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 Pediat, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Psychiat, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 ychiat, Baltimore, MD 21205 USA Kennedy Krieger Inst, Neurogenet Unit, Baltimore, MD USA Kennedy Krieger Inst Baltimore MD USA Neurogenet Unit, Baltimore, MD USA
Titolo Testata:
NEUROLOGY
fascicolo: 3, volume: 54, anno: 2000,
pagine: 715 - 722
SICI:
0028-3878(20000208)54:3<715:QHMSII>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
MAGNETIC-RESONANCE SPECTROSCOPY; TEMPORAL-LOBE EPILEPSY; CEREBRAL BLOOD-FLOW; HUMAN BRAIN; CORTEX; ABNORMALITIES; EXPRESSION; METABOLISM; NEOCORTEX; CRITERIA;
Keywords:
Rett syndrome; brain metabolism; magnetic resonance spectroscopy;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
39
Recensione:
Indirizzi per estratti:
Indirizzo: Horska, A Johns Hopkins Univ, Sch Med, Dept Radiol, 217 Traylor Bldg,720 Rutland Ave, Baltimore, MD 21205 USA Johns Hopkins Univ 217 Traylor Bldg,720Rutland Ave Baltimore MD USA 21205
Citazione:
A. Horska et al., "Quantitative H-1 MR spectroscopic imaging in early Rett syndrome", NEUROLOGY, 54(3), 2000, pp. 715-722

Abstract

Objective: To determine cerebral regional concentrations of N-acetyl aspartate (NAA), total choline (Cho), and total creatine (Cr) in Rett syndrome (RS) using H-1 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 H-1 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 < 0.005) and average NAA concentration 11% lower (p < 0.0001) compared with the control group. Regional metabolic differences included significantly lower NAA concentration in the frontal gray and white matter, insula, and hippocampus in RS; no difference in regional Cho and Cr concentrations were found. A 20 to 38% higher Cho:NAA ratio in frontal and parietal gray and white matter, insular gray matter,and hippocampus (p < 0.05) and a 14 to 47% lower NAA:Cr ratio in frontal cortical gray matter, parietal and temporal white matter, insula, and putamen (p < 0.05) were found in subjects with RS compared with controls. Patients with seizures had higher average concentrations of Cho, Cr, and NAA compared with those without seizures (8-19%, p < 0.05). 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.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/01/20 alle ore 16:02:23