Diffusion tensor imaging and brain volumetry in Fabry disease patients

Autor: Ronald Borra, Jani Saunavaara, Virva Lepomäki, Teemu Paavilainen, Pirjo Nuutila, Ilkka Kantola, Riitta Parkkola
Rok vydání: 2013
Předmět:
Male
Pathology
OUTCOME SURVEY
ROBUST
CEREBRAL ATROPHY
computer.software_genre
Nerve Fibers
Myelinated
Tract-based spatial statistics
Voxel
Cerebrovascular disease
Brain
Organ Size
Middle Aged
WHITE-MATTER LESIONS
Diffusion-tensor imaging
HYPERINTENSITIES
MANIFESTATIONS
medicine.anatomical_structure
REGISTRATION
MR-IMAGES
Female
Cardiology and Cardiovascular Medicine
Adult
medicine.medical_specialty
Adolescent
Grey matter
Sensitivity and Specificity
White matter
Young Adult
Imaging
Three-Dimensional
Image Interpretation
Computer-Assisted
Fractional anisotropy
medicine
Humans
Radiology
Nuclear Medicine and imaging
OPTIMIZATION
SCLEROSIS
Aged
Cerebral atrophy
Fabry disease
business.industry
Reproducibility of Results
medicine.disease
Metabolic abnormalities
Hyperintensity
Diffusion Magnetic Resonance Imaging
Neurology (clinical)
business
Nuclear medicine
computer
Diffusion MRI
Zdroj: Neuroradiology, 55(5), 551-558. SPRINGER
ISSN: 1432-1920
0028-3940
Popis: Fabry disease is a rare lysosomal storage disorder leading to cellular accumulation of globotriaosylceramide, especially in blood vessels. It is associated with severe early onset cerebrovascular disease and kidney and heart failure. The purpose of this study was to reveal possible disturbances in white matter integrity in Fabry disease patients using voxelwise diffusion-tensor imaging (DTI) analysis.Twelve Fabry disease patients, along with 13 healthy controls, underwent DTI and structural MRI. Voxel-based analysis of the DTI data was performed to assess possible differences in DTI parameters between Fabry disease patients and healthy controls. A selective region of interest analysis was performed for healthy volunteers and Fabry disease patients having a mild burden of T2-hyperintense lesions. We also measured normalised brain tissue volumes and performed a voxel-based volume analysis for grey matter.Voxel-based analysis of DTI data showed areas of significantly reduced fractional anisotropy and increased mean diffusivity in patients with Fabry disease. Eight patients had a mild burden of white matter lesions on their T2 scans. Region of interest analysis on areas showing reduced fractional anisotropy in voxelwise analysis also revealed reduced fractional anisotropy values in this patient group compared to eight healthy volunteers. The brain volume analyses did not reveal significant differences between the Fabry disease patients and the controls.These findings suggest a microstructural damage in brain white matter of Fabry disease patients, which can be revealed before excessive white matter lesions load is visible on conventional MR scans.
Databáze: OpenAIRE
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