In Vivo Assessment of Neurodegeneration in Type C Niemann-Pick Disease by IDEAL-IQ
- Author:
Ruo Mi GUO
1
;
Qing Ling LI
;
Zhong Xing LUO
;
Wen TANG
;
Ju JIAO
;
Jin WANG
;
Zhuang KANG
;
Shao Qiong CHEN
;
Yong ZHANG
Author Information
- Publication Type:Brief Communication
- Keywords: Neurodegeneration; Magnetic resonance imaging; Magnetic resonance spectroscopy; Niemann-Pick type C disease
- MeSH: Brain; Female; Follow-Up Studies; Healthy Volunteers; Humans; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Male; Methods; Niemann-Pick Diseases; Proton Magnetic Resonance Spectroscopy; Water
- From:Korean Journal of Radiology 2018;19(1):93-100
- CountryRepublic of Korea
- Language:English
- 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 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.
