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KMID : 0880420180190010093
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Korean Journal of Radiology
2018 Volume.19 No. 1 p.93 ~ p.100
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In Vivo Assessment of Neurodegeneration in Type C Niemann-Pick Disease by IDEAL-IQ
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Guo Ruo-Mi
Li Qing-Ling
Luo Zhong-Xing
Tang Wen
Jiao Ju
Wang Jin
Kang Zhuang
Chen Shao-Qiong
Zhang Yong
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Abstract
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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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KEYWORD
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Neurodegeneration, Magnetic resonance imaging, Magnetic resonance spectroscopy, Niemann-Pick type C disease
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