Tract-based evaluation of white matter damage in individuals with early-treated phenylketonuria.
J Inherit Metab Dis
; 37(2): 237-43, 2014 Mar.
Article
en En
| MEDLINE
| ID: mdl-24043380
ABSTRACT
Previous research has documented white matter abnormalities in the brains of individuals with early-treated phenylketonuria (ETPKU). The majority of these past studies have relied on a region-based approach which focused on a limited number of spatially-defined regions within the brain. In the present study, we used diffusion tensor imaging (DTI) in conjunction with tract-based spatial statistics (TBSS) to perform an extensive examination of white matter tracts in the brains of ten individuals with ETPKU (mean age = 23.2 years) and 12 healthy non-PKU individuals (mean age = 23.5 years). Consistent with past research, we found that mean diffusivity (MD) was significantly restricted in the ETPKU group, and fractional anisotropy (FA) was comparable between the ETPKU and non-PKU groups. Moreover, we found restricted axial diffusivity (AD) and radial diffusivity (RD) in our ETPKU in numerous white matter tracts, suggesting widespread white matter compromise in ETPKU. In addition, this white matter pathology was more evident in older ETPKU participants with higher blood phenylalanine (phe) levels as compared to younger participants with lower phe levels.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Fenilcetonurias
/
Sustancia Blanca
Tipo de estudio:
Observational_studies
Límite:
Adolescent
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Adult
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Child
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Female
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Humans
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Male
Idioma:
En
Revista:
J Inherit Metab Dis
Año:
2014
Tipo del documento:
Article
País de afiliación:
Estados Unidos