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      저자 : Ruo-Mi Guo (검색결과 1 건)
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        In Vivo Assessment of Neurodegeneration in Type C Niemann-Pick Disease by IDEAL-IQ

        Ruo-Mi Guo,Qing-Ling Li,Zhong-Xing Luo,Wen Tang,Ju Jiao,Jin Wang,Zhuang Kang,Shao-Qiong Chen,Yong Zhang 대한영상의학회 2018 Korean Journal of Radiology Vol.19 No.1

        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.

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