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Yonsei medical journal v.51 no.5, 2010년, pp.672 - 675   SCIE
본 등재정보는 저널의 등재정보를 참고하여 보여주는 베타서비스로 정확한 논문의 등재여부는 등재기관에 확인하시기 바랍니다.

Initial Experiences with Proton MR Spectroscopy in Treatment Monitoring of Mitochondrial Encephalopathy

Lee, Seung-Koo    (Department of Radiology, Yonsei University College of Medicine, Seoul, Korea.   ); Kim, Jinna    (Department of Radiology, Yonsei University College of Medicine, Seoul, Korea.   ); Kim, Heung Dong    (Department of Pediatric Neurology, Yonsei University College of Medicine, Seoul, Korea.   ); Lee, Joon Soo    (Department of Pediatric Neurology, Yonsei University College of Medicine, Seoul, Korea.   ); Lee, Young Mock    (Department of Pediatric Neurology, Yonsei University College of Medicine, Seoul, Korea.  );
  • 초록

    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 1 H-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 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.


  • 주제어

    Magnetic resonance spectroscopy .   mitochondrial diseases.  

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