In Vivo Assessment of Neurodegeneration in Type C Niemann-Pick Disease by IDEAL-IQ
Korean Journal of Radiology
;
: 93-100, 2018.
Artigo
em Inglês
| WPRIM
| ID: wpr-741380
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 ANDMETHODS:
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Encéfalo
/
Imageamento por Ressonância Magnética
/
Água
/
Espectroscopia de Ressonância Magnética
/
Doenças de Niemann-Pick
/
Seguimentos
/
Voluntários Saudáveis
/
Espectroscopia de Prótons por Ressonância Magnética
/
Métodos
Tipo de estudo:
Estudo observacional
/
Estudo prognóstico
Limite:
Feminino
/
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Korean Journal of Radiology
Ano de publicação:
2018
Tipo de documento:
Artigo
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