Literature DB >> 24059666

Proton MR Spectroscopy in Patients with Leigh Syndrome.

E Jurkiewicz1, S Chełstowska, I Pakuła-Kościesza, K Malczyk, K Nowak, M Bekiesińska-Figatowska, J Sykut-Cegielska, D Piekutowska-Abramczuk, E Pronicka.   

Abstract

The aim of the present study was to evaluate MRS findings in patients with Leigh syndrome. We report our results of HMR spectroscopic studies performed in six patients (aged four months to ten years) with clinically proved Leigh syndrome. All examinations were done with 1.5 T scanner using an eight-channel phased array head coil. HMRS data were obtained using 2D-chemical shift imaging (CSI) and SVS sequences with short (30 ms) and long (135 ms) echo time. The MR spectra were acquired in multiple voxel localized in deep gray matter and periventricular white matter. The results were compared to the control group data. In most of our patients we found bilateral lesions in the basal ganglia and brain stem. HMRS data revealed elevated lactate in the affected areas, significantly diminished NAA/Cr ratio. The relatively high Cho/Cr ratio in the gray and white matter was also noted. HMRS is an important tool for non-invasive brain tissue analysis in Leigh syndrome.

Entities:  

Year:  2011        PMID: 24059666     DOI: 10.1177/197140091102400312

Source DB:  PubMed          Journal:  Neuroradiol J        ISSN: 1971-4009


  3 in total

1.  Contribution of the MRPS22 variant and a Down mosaic to the phenotype.

Authors:  Josef Finsterer; Sinda Zarrouk-Mahjoub
Journal:  Metab Brain Dis       Date:  2017-09-30       Impact factor: 3.584

2.  Lesional perfusion abnormalities in Leigh disease demonstrated by arterial spin labeling correlate with disease activity.

Authors:  Matthew T Whitehead; Bonmyong Lee; Andrea Gropman
Journal:  Pediatr Radiol       Date:  2016-04-04

3.  Why does Leigh syndrome respond to immunotherapy?

Authors:  Josef Finsterer; Sinda Zarrouk-Mahjoub
Journal:  Mol Genet Metab Rep       Date:  2016-07-27
  3 in total

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