BRAIN: Is white matter integrity associated with cognition and disease severity in patients with Fabry disease?

Cerebral whiteness pathology is a common manifestation of the central nervous system of Fabry disease, with 42-81% of patients with MRI as a high signal of whiteness.
Spectre Imaging (DTI) MRI is a sensitive technique for quantitative microstructural damage in white matter, with potential disease biomarker value.
here, the researchers assessed patterns of DTI abnormalities in Fabri's disease and their correlation with cognitive impairment, mood, anxiety, severity of the disease and plasma-soluble Gb3 levels in 31 genetically proven Fabri disease and 19 age-matched healthy control subjects.
study obtained an average of the fractional an opposite sex and average diffusion rate in white mass, and used region-based spatial statistics for physical analysis.
used standardized neuropsychological test batteries to assess processing speed, executive function, anxiety, depression and severity of the disease.
age (percentage of males) was 44.1 (45%) for patients with Fabri disease and 37.4 (53%) for the health control group.
compared to the health control group, the average average white mass score of patients with Fabbri's disease was lower than that of the average white mass score of 0.423 (standard deviation, SD 0.023) to 0.446 (SD 0.016), P was 0.002, while the average white mass diffusion was average. Higher rates (749×10-6 mm2 / s (SD 32×10-6) and 720×10-6 mm2 / s (SD 21×10-6), P s 0.004.
Voxelwise statistics show that the two fractions of antonym diffusion abnormal lesions probability graph shows that the white high signal also has a wide anatomical distribution, and biased towards the rear center half follicles, but the spread of Fabry disease abnormalities are not limited to diseased tissue; 3 (SD 0.017) P s 0.003, the average diffusion rate increased by 747×10-6 mm2 / s (SD 26×10-6) to 723× 10-6 mm2 / s (SD 22×10-6), P to 0.008.
In patients, the average white mass score anthopathicity and whiteness lesions volume showed a statistically significant correlation (r s 0.558, P s 0.001; r s -0.633, P≤0.001).
The anthoidity of the average white matter score was associated with the overall Mainz severity score index (r - 0.661, P≤0.001), while the average average white matter diffusion rate was strongly corred with the plasma-soluble Gb3 level (r s 0.559, P s 0.001).
the use of DTI confirms that the wide range of microstructural whiteness damage in Fabri's disease exceeds the high whiteness signal seen in conventional MRIs.
In addition, dispersion measurements are highly relevant to hypothetical plasma biomarkers of cognitive (processing speed), clinical disease severity, and disease activity, and are therefore expected to be promising quantitative biomarkers for monitoring the severity and progression of Fabry disease.
Original source: Leonardo Ulivi, Baris Kanber, Ferran Prados, MedSci Original Copyright Notice: All text, images and audio and video materials on this website that indicate "Source: Mets Medicine" or "Source: MedSci Originals" are owned by Metz Medicine and are not authorized by any media, website or individual to be reproduced.
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