Subcortical Damage and Cortical Functional Changes in Men and Women with Fabry Disease: A Multifaceted MR Study
Autor: | Natale Villari, Cinzia Gavazzi, Walter Borsini, Massimo Filippi, Riccardo Della Nave, Maria A. Rocca, Suzanne Buchner, Laura Guerrini, Giacomo Belli, Mario Mascalchi, Carlo Tessa |
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Přispěvatelé: | Gavazzi, C, Borsini, W, Guerrini, L, Della Nave, R, Rocca, Ma, Tessa, C, Buchner, S, Belli, G, Filippi, Massimo, Villari, N, Mascalchi, M. |
Rok vydání: | 2006 |
Předmět: |
Adult
Male In vivo magnetic resonance spectroscopy medicine.medical_specialty Magnetic Resonance Spectroscopy Statistical parametric mapping Statistics Nonparametric White matter medicine Humans Radiology Nuclear Medicine and imaging Prospective Studies Prospective cohort study business.industry Vascular disease Case-control study Brain medicine.disease Control subjects Magnetic Resonance Imaging Fabry disease Surgery medicine.anatomical_structure Case-Control Studies Linear Models Fabry Disease Female Radiology business |
Zdroj: | Radiology. 241:492-500 |
ISSN: | 1527-1315 0033-8419 |
DOI: | 10.1148/radiol.2412051122 |
Popis: | To prospectively compare brain magnetic resonance (MR) imaging and hydrogen 1 (1H) MR spectroscopy findings and to use functional MR imaging to explore the patterns of brain activation in men and women with Fabry disease (FD).Eight men and eight women with FD (mean age, 38.8 years +/- 13.9 [standard deviation]) with absent or mild neurologic deficit and 16 healthy control subjects (eight men and eight women; mean age, 42.7 years +/- 15.3) gave informed consent to participate in the study, which was approved by the local ethical committee. Patients and control subjects underwent MR imaging, 1H MR spectroscopy of the frontal cortex and subcortical white matter, and functional MR imaging during repetitive flexion-extension of the last four fingers of the right hand. Extent of cerebral white matter damage was rated on fluid-attenuated inversion recovery MR images by using a visual score. Areas of activation were identified by using statistical parametric mapping software and the adoption of a height threshold of P.001 (uncorrected) and an extent threshold of P.05 (corrected).Men and women with FD showed a similar distribution of cerebral white matter changes, lacunar and cortical infarcts, small hemorrhages, and vertebrobasilar dolichoectasia. No significant (P.05) difference was observed between patients with FD and control subjects for concentration of N-acetylaspartate, creatine, and choline. During the motor task, patients showed recruitment of additional cortical areas in comparison with control subjects. Increased activation of the contralateral sensorimotor area correlated (P = .002) with extent of white matter damage.Subcortical ischemic changes in men and women with FD are similar and are associated with increased recruitment of the sensorimotor network during a simple motor task, which might limit the functional effect of the white matter small-vessel disease. |
Databáze: | OpenAIRE |
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