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Initial experiences with proton MR spectroscopy in treatment monitoring of mitochondrial encephalopathy.

Lee SK, Kim J, Kim HD, Lee JS, Lee YM - Yonsei Med. J. (2010)

Bottom Line: Lactate peak was found in 4 patients; 2 had disappearance of the peak on follow up MRS.Quantitative analysis showed relative decrease of Cho/Cr ratio on follow up MRS (p = 0.0058, paired t-test, two-tailed).There was no significant change in NAA/Cr ratio.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Yonsei University College of Medicine, 250 Seongsan-ro, Seodaemun-gu, Seoul 120-752, Korea. slee@yuhs.ac

ABSTRACT

Purpose: Mitochondrial encephalopathy (ME) is a rare disorder of energy metabolism. The disease course can roughly be evaluated by clinical findings. The purpose of this study was to evaluate metabolic spectral changes using proton MR spectroscopy (MRS), and to establish a way to monitor ME by neuroimaging.

Materials and methods: Proton MRS data were retrospectively reviewed in 12 patients with muscle biopsy-confirmed ME (M : F = 7 : 5, Mean age = 4.8 years). All received 1H-MRS initially and also after a ketogenic diet and mitochondrial disease treatment cocktail (follow up average was 10.2 months). Changes of N-acetylaspartate/ creatine (NAA/Cr) ratio, choline/creatine (Cho/Cr) ratio, and lactate peak in basal ganglia at 1.2 ppm were evaluated before and after treatment. Findings on conventional T2 weighted MR images were also evaluated.

Results: On conventional MRI, increased basal ganglia T2 signal intensity was the most common finding with ME (n = 9, 75%), followed by diffuse cerebral atrophy (n = 8, 67%), T2 hyperintense lesions at pons and midbrain (n = 4, 33%), and brain atrophy (n = 2, 17%). Lactate peak was found in 4 patients; 2 had disappearance of the peak on follow up MRS. Quantitative analysis showed relative decrease of Cho/Cr ratio on follow up MRS (p = 0.0058, paired t-test, two-tailed). There was no significant change in NAA/Cr ratio.

Conclusion: MRS is a useful tool for monitoring disease progression or improvement in ME, and decrease or disappearance of lactate peak and reduction of Cho/Cr fraction were correlated well with improvement of clinical symptoms.

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Related in: MedlinePlus

Two-year-old male with Leigh disease (case 2). Pre-treatment MRS shows high lactate peak (arrow) at basal ganglia. One year follow up MRS shows decrease of lactate (double arrows) and restoration of NAA. NAA, N-acetylaspartate.
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Figure 1: Two-year-old male with Leigh disease (case 2). Pre-treatment MRS shows high lactate peak (arrow) at basal ganglia. One year follow up MRS shows decrease of lactate (double arrows) and restoration of NAA. NAA, N-acetylaspartate.

Mentions: On conventional T2 weighted images (T2WI), all had abnormal findings. Increased basal ganglia T2 signal intensity was the most common finding with ME (n = 9, 75%), followed by diffuse cerebral atrophy (n = 8, 67%). Four (33%) patients had increased midbrain and pons signal lesions. Cerebellar atrophy (17%) was found in 2 patients, along with diffuse cerebral atrophy (Table 1). Lactate peak was found in 4 patients; 2 had disappearance of the peak on follow up scan. One patient showed marked reduction of lactate peak after ketogenic diet and mitochondrial cocktail therapy (Case #2, Fig. 1), but the other patient showed no remarkable change of lactate peak before and after treatment.


Initial experiences with proton MR spectroscopy in treatment monitoring of mitochondrial encephalopathy.

Lee SK, Kim J, Kim HD, Lee JS, Lee YM - Yonsei Med. J. (2010)

Two-year-old male with Leigh disease (case 2). Pre-treatment MRS shows high lactate peak (arrow) at basal ganglia. One year follow up MRS shows decrease of lactate (double arrows) and restoration of NAA. NAA, N-acetylaspartate.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2908880&req=5

Figure 1: Two-year-old male with Leigh disease (case 2). Pre-treatment MRS shows high lactate peak (arrow) at basal ganglia. One year follow up MRS shows decrease of lactate (double arrows) and restoration of NAA. NAA, N-acetylaspartate.
Mentions: On conventional T2 weighted images (T2WI), all had abnormal findings. Increased basal ganglia T2 signal intensity was the most common finding with ME (n = 9, 75%), followed by diffuse cerebral atrophy (n = 8, 67%). Four (33%) patients had increased midbrain and pons signal lesions. Cerebellar atrophy (17%) was found in 2 patients, along with diffuse cerebral atrophy (Table 1). Lactate peak was found in 4 patients; 2 had disappearance of the peak on follow up scan. One patient showed marked reduction of lactate peak after ketogenic diet and mitochondrial cocktail therapy (Case #2, Fig. 1), but the other patient showed no remarkable change of lactate peak before and after treatment.

Bottom Line: Lactate peak was found in 4 patients; 2 had disappearance of the peak on follow up MRS.Quantitative analysis showed relative decrease of Cho/Cr ratio on follow up MRS (p = 0.0058, paired t-test, two-tailed).There was no significant change in NAA/Cr ratio.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Yonsei University College of Medicine, 250 Seongsan-ro, Seodaemun-gu, Seoul 120-752, Korea. slee@yuhs.ac

ABSTRACT

Purpose: Mitochondrial encephalopathy (ME) is a rare disorder of energy metabolism. The disease course can roughly be evaluated by clinical findings. The purpose of this study was to evaluate metabolic spectral changes using proton MR spectroscopy (MRS), and to establish a way to monitor ME by neuroimaging.

Materials and methods: Proton MRS data were retrospectively reviewed in 12 patients with muscle biopsy-confirmed ME (M : F = 7 : 5, Mean age = 4.8 years). All received 1H-MRS initially and also after a ketogenic diet and mitochondrial disease treatment cocktail (follow up average was 10.2 months). Changes of N-acetylaspartate/ creatine (NAA/Cr) ratio, choline/creatine (Cho/Cr) ratio, and lactate peak in basal ganglia at 1.2 ppm were evaluated before and after treatment. Findings on conventional T2 weighted MR images were also evaluated.

Results: On conventional MRI, increased basal ganglia T2 signal intensity was the most common finding with ME (n = 9, 75%), followed by diffuse cerebral atrophy (n = 8, 67%), T2 hyperintense lesions at pons and midbrain (n = 4, 33%), and brain atrophy (n = 2, 17%). Lactate peak was found in 4 patients; 2 had disappearance of the peak on follow up MRS. Quantitative analysis showed relative decrease of Cho/Cr ratio on follow up MRS (p = 0.0058, paired t-test, two-tailed). There was no significant change in NAA/Cr ratio.

Conclusion: MRS is a useful tool for monitoring disease progression or improvement in ME, and decrease or disappearance of lactate peak and reduction of Cho/Cr fraction were correlated well with improvement of clinical symptoms.

Show MeSH
Related in: MedlinePlus