TY - JOUR
T1 - Subcortical damage and cortical functional changes in men and women with fabry disease
T2 - A multifaceted MR study
AU - Gavazzi, Cinzia
AU - Borsini, Walter
AU - Guerrini, Laura
AU - Della Nave, Riccardo
AU - Rocca, Maria Assunta
AU - Tessa, Carlo
AU - Buchner, Suzanne
AU - Belli, Giacomo
AU - Filippi, Massimo
AU - Villari, Natale
AU - Mascalchi, Mario
PY - 2006/11
Y1 - 2006/11
N2 - Purpose: 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). Materials and Methods: 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). Results: 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. Conclusion: 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.
AB - Purpose: 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). Materials and Methods: 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). Results: 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. Conclusion: 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.
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U2 - 10.1148/radiol.2412051122
DO - 10.1148/radiol.2412051122
M3 - Article
C2 - 17057070
AN - SCOPUS:33750366055
SN - 0033-8419
VL - 241
SP - 492
EP - 500
JO - Radiology
JF - Radiology
IS - 2
ER -