Abstract
Background: Analysis of serum biomarkers and magnetic resonance spectroscopy (MRS) are useful for monitoring disease progression in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). We evaluated the correlation of serum biomarkers and MRS parameters during changes associated with stroke-like episodes.Methods: In 13 symptomatic MELAS patients carrying the A3243G mutation, we retrospectively obtained 207 voxels from 41 MRS studies, which were divided into three groups according to the temporal association with stroke-like episodes. The MRS NAA/Cr, Cho/Cr, NAA/Cho ratios, the presence of a lactate peak, serum biomarkers, serum lactate level and the pyruvate (Lac/Pyr) ratio were determined.Results: In regions with acute infarcts, the severity of serum Lac/Pyr and that of the MRS lactate peak (P = 0.0007) correlated; serum lactate (P = 0.02), severity of elevated serum lactate (P = 0.04), and serum Lac/Pyr (P = 0.02) correlated weakly. In previously infarcted regions, the severity of the MRS lactate peak and serum Lac/Pyr (P = 0.03), as well as the severity of serum Lac/Pyr (P = 0.02) were weakly correlated. In structurally normal regions, we found a weak to moderate negative correlation between serum lactate and MRS NAA/Cr (P = 0.008), and between the severity of elevated serum lactate and MRS NAA/Cr (P = 0.002) as well as MRS NAA/Cho (P = 0.02).Conclusions: MRS parameters correlate with specific serum biomarkers, and are useful for monitoring changes in brain metabolites, particularly as related to stroke-like episodes.
Highlights
Mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome is characterized by its unique features of stroke-like episodes and associated neurological symptoms and psychomotor regression [1, 2], related to defective mitochondrial energy metabolism [3, 4]
We found a weak to moderate negative correlation between serum lactate and magnetic resonance spectroscopy (MRS) N-acetylaspartate/creatine ratio (NAA/Cr) (P = 0.008), and between the severity of elevated serum lactate and MRS NAA/Cr (P = 0.002) as well as MRS Nacetylaspartate/choline ratio (NAA/Cho) (P = 0.02)
According to the recent diagnostic criteria reported by Yatsuga et al [8], acute focal lesions seen on brain images, such as magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS), are common in MELAS
Summary
Mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome is characterized by its unique features of stroke-like episodes and associated neurological symptoms and psychomotor regression [1, 2], related to defective mitochondrial energy metabolism [3, 4]. According to the recent diagnostic criteria reported by Yatsuga et al [8], acute focal lesions seen on brain images, such as magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS), are common in MELAS These changes occur in relation to stroke-like episodes and vary, but occur outside the usual territories of vascular infarction [1, 9,10,11,12]. Analysis of serum biomarkers and magnetic resonance spectroscopy (MRS) are useful for monitoring disease progression in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). We evaluated the correlation of serum biomarkers and MRS parameters during changes associated with stroke-like episodes
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