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.
journal_name
Yonsei Med Jjournal_title
Yonsei medical journalauthors
Lee SK,Kim J,Kim HD,Lee JS,Lee YMdoi
10.3349/ymj.2010.51.5.672subject
Has Abstractpub_date
2010-09-01 00:00:00pages
672-5issue
5eissn
0513-5796issn
1976-2437pii
201009672journal_volume
51pub_type
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journal_title:Yonsei medical journal
pub_type: 杂志文章
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更新日期:2007-10-31 00:00:00
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journal_title:Yonsei medical journal
pub_type: 杂志文章,收录出版
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journal_title:Yonsei medical journal
pub_type: 杂志文章
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journal_title:Yonsei medical journal
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journal_title:Yonsei medical journal
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journal_title:Yonsei medical journal
pub_type: 杂志文章
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journal_title:Yonsei medical journal
pub_type: 杂志文章
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journal_title:Yonsei medical journal
pub_type: 杂志文章,评审
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journal_title:Yonsei medical journal
pub_type: 杂志文章
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journal_title:Yonsei medical journal
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pub_type: 杂志文章
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更新日期:2003-06-30 00:00:00
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journal_title:Yonsei medical journal
pub_type: 杂志文章
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更新日期:2016-01-01 00:00:00
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journal_title:Yonsei medical journal
pub_type: 杂志文章
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pub_type: 杂志文章
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journal_title:Yonsei medical journal
pub_type: 杂志文章
doi:10.3349/ymj.1999.40.5.478
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journal_title:Yonsei medical journal
pub_type: 已发布勘误
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journal_title:Yonsei medical journal
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journal_title:Yonsei medical journal
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doi:10.3349/ymj.2006.47.6.877
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journal_title:Yonsei medical journal
pub_type: 杂志文章
doi:10.3349/ymj.2004.45.5.944
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journal_title:Yonsei medical journal
pub_type: 杂志文章
doi:10.3349/ymj.2005.46.5.700
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pub_type: 杂志文章
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journal_title:Yonsei medical journal
pub_type: 杂志文章
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更新日期:2007-12-31 00:00:00
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journal_title:Yonsei medical journal
pub_type: 杂志文章
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