Assessment of Neurodegeneration in Type C Niemann-Pick Disease by IDEAL-IQ

Overview

Journal Korean J Radiol

Specialty Radiology

Date 2018 Jan 23

PMID 29354005

Citations 3

Authors

Ruo-Mi Guo

Qing-Ling Li

Zhong-Xing Luo

Wen Tang

Ju Jiao

Jin Wang

Zhuang Kang

Shao-Qiong Chen

Yong Zhang

Affiliations

Soon will be listed here.

Abstract

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 H-proton magnetic resonance spectroscopy (H-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 H-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%, < 0.05 and 1.8-3.0%, < 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 ( < 0.05). The results of the H-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 H-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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