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

Seung Koo Lee, Jinna Kim, Heung Dong Kim, Joon Soo Lee, Young Mock Lee

Research output: Contribution to journalReview article

13 Citations (Scopus)

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 Nacetylaspartate/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 impro-vement in ME, and decrease or disappearance of lactate peak and reduction of Cho/Cr fraction were correlated well with improvement of clinical symptoms.

Original languageEnglish
Pages (from-to)672-675
Number of pages4
JournalYonsei medical journal
Volume51
Issue number5
DOIs
Publication statusPublished - 2010 Sep

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Creatine
Protons
Magnetic Resonance Spectroscopy
Choline
Lactic Acid
Basal Ganglia
Atrophy
Therapeutics
Ketogenic Diet
Mitochondrial Diseases
Pons
Mesencephalon
Neuroimaging
Energy Metabolism
Disease Progression
Mitochondrial encephalopathy
Biopsy
Muscles
Brain

All Science Journal Classification (ASJC) codes

  • Medicine(all)

Cite this

Lee, Seung Koo ; Kim, Jinna ; Kim, Heung Dong ; Lee, Joon Soo ; Lee, Young Mock. / Initial experiences with proton MR spectroscopy in treatment monitoring of mitochondrial encephalopathy. In: Yonsei medical journal. 2010 ; Vol. 51, No. 5. pp. 672-675.
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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 Nacetylaspartate/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 impro-vement 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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Initial experiences with proton MR spectroscopy in treatment monitoring of mitochondrial encephalopathy. / Lee, Seung Koo; Kim, Jinna; Kim, Heung Dong; Lee, Joon Soo; Lee, Young Mock.

In: Yonsei medical journal, Vol. 51, No. 5, 09.2010, p. 672-675.

Research output: Contribution to journalReview article

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T1 - Initial experiences with proton MR spectroscopy in treatment monitoring of mitochondrial encephalopathy

AU - Lee, Seung Koo

AU - Kim, Jinna

AU - Kim, Heung Dong

AU - Lee, Joon Soo

AU - Lee, Young Mock

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N2 - 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 Nacetylaspartate/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 impro-vement in ME, and decrease or disappearance of lactate peak and reduction of Cho/Cr fraction were correlated well with improvement of clinical symptoms.

AB - 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 Nacetylaspartate/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 impro-vement 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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