Korean Journal of Radiology

  • 제19권1호
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  • Pages.93-100
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  • 2018
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  • 1229-6929(pISSN)
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  • 2005-8330(eISSN)
대한영상의학회 (The Korean Society of Radiology)
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In Vivo Assessment of Neurodegeneration in Type C Niemann-Pick Disease by IDEAL-IQ

  • Guo, Ruo-Mi (Department of Radiology, the Third Affiliated Hospital, Sun Yat-Sen University) ;
  • Li, Qing-Ling (Department of VIP Medical Center, the Third Affiliated Hospital, Sun Yat-Sen University) ;
  • Luo, Zhong-Xing (Department of Radiology, the Third Affiliated Hospital, Sun Yat-Sen University) ;
  • Tang, Wen (Department of Radiology, the Third Affiliated Hospital, Sun Yat-Sen University) ;
  • Jiao, Ju (Department of Nuclear Medicine, the Third Affiliated Hospital, Sun Yat-Sen University) ;
  • Wang, Jin (Department of Radiology, the Third Affiliated Hospital, Sun Yat-Sen University) ;
  • Kang, Zhuang (Department of Radiology, the Third Affiliated Hospital, Sun Yat-Sen University) ;
  • Chen, Shao-Qiong (Department of Radiology, the Third Affiliated Hospital, Sun Yat-Sen University) ;
  • Zhang, Yong (Department of Radiology, the Third Affiliated Hospital, Sun Yat-Sen University)
  • 투고 : 2016.11.15
  • 심사 : 2017.01.01
  • 발행 : 2018.02.01
⟨ 이전 논문 다음 논문 ⟩

초록

Objective: To noninvasively assess the neurodegenerative changes in the brain of patients with Niemann-Pick type C (NPC) disease by measuring the lesion tissue with the iterative decomposition of water and fat with echo asymmetry and least square estimation-iron quantification (IDEAL-IQ). Materials and Methods: Routine brain MRI, IDEAL-IQ and $^1H-proton$ magnetic resonance spectroscopy ($^1H-MRS$, served as control) were performed on 12 patients with type C Niemann-Pick disease (4 males and 8 females; age range, 15-61 years; mean age, 36 years) and 20 healthy subjects (10 males and 10 females; age range, 20-65 years; mean age, 38 years). The regions with lesion and the normal appearing regions (NARs) of patients were measured and analyzed based on the fat/water signal intensity on IDEAL-IQ and the lipid peak on $^1H-MRS$. Results: Niemann-Pick type C patients showed a higher fat/water signal intensity ratio with IDEAL-IQ on T2 hyperintensity lesions and NARs (3.7-4.9%, p < 0.05 and 1.8-3.0%, p < 0.05, respectively), as compared to healthy controls (HCs) (1.2-2.3%). After treatment, the fat/water signal intensity ratio decreased (2.2-3.4%), but remained higher than in the HCs (p < 0.05). The results of the $^1H-MRS$ measurements showed increased lipid peaks in the same lesion regions, and the micro-lipid storage disorder of NARs in NPC patients was detectable by IDEAL-IQ instead of $^1H-MRS$. Conclusion: The findings of this study suggested that IDEAL-IQ may be useful as a noninvasive and objective method in the evaluation of patients with NPC; additionally, IDEAL-IQ can be used to quantitatively measure the brain parenchymal adipose content and monitor patient follow-up after treatment of NPC.

키워드

과제정보

연구 과제 주관 기관 : Medical Science Foundation of Guangdong Provence

참고문헌

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