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Proton MR Spectroscopy in Patients with Leigh Syndrome.
E Jurkiewicz1, S Chełstowska, I Pakuła-Kościesza
1Magnetic Resonance Department; Children's Memorial Health Institute; Warsaw, Poland - e-jurkiewicz@o2.pl.
The Neuroradiology Journal
|September 25, 2013
Summary
Proton magnetic resonance spectroscopy (MRS) reveals elevated lactate and altered metabolite ratios in patients with Leigh syndrome, aiding in non-invasive brain tissue analysis. This study highlights MRS as a crucial diagnostic tool for this rare neurological disorder.
Area of Science:
- Neuroscience
- Medical Imaging
- Biochemistry
Background:
- Leigh syndrome is a severe, progressive neurodegenerative disorder affecting infants and children.
- Accurate diagnosis and monitoring of Leigh syndrome are crucial for patient management.
- Conventional MRI may show non-specific findings, necessitating advanced imaging techniques.
Purpose of the Study:
- To evaluate the utility of magnetic resonance spectroscopy (MRS) in characterizing brain abnormalities in Leigh syndrome.
- To identify specific metabolic alterations detectable by MRS in affected individuals.
- To compare MRS findings in Leigh syndrome patients with healthy controls.
Main Methods:
- Six patients diagnosed with Leigh syndrome underwent 1.5 T magnetic resonance (MR) imaging.
- Proton MRS was performed using 2D-chemical shift imaging (CSI) and single-voxel spectroscopy (SVS) sequences.
- MR spectra were acquired from deep gray matter and periventricular white matter, with short and long echo times, and compared to control data.
Main Results:
- Bilateral lesions in the basal ganglia and brain stem were observed in most patients.
- MRS data demonstrated elevated lactate levels in affected brain regions.
- A significantly diminished N-acetylaspartate/creatine (NAA/Cr) ratio and a relatively high choline/creatine (Cho/Cr) ratio were noted.
Conclusions:
- Proton magnetic resonance spectroscopy (MRS) is a valuable non-invasive tool for brain tissue analysis in Leigh syndrome.
- Characteristic metabolic changes, including elevated lactate and altered NAA/Cr and Cho/Cr ratios, support the diagnosis of Leigh syndrome.
- MRS findings can aid in the early detection and monitoring of disease progression in Leigh syndrome.
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