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Subcortical damage and cortical functional changes in men and women with Fabry disease: a multifaceted MR study.

Abstract：

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.

journal_name

Radiology

journal_title

Radiology

authors

Gavazzi C,Borsini W,Guerrini L,Della Nave R,Rocca MA,Tessa C,Buchner S,Belli G,Filippi M,Villari N,Mascalchi M

doi

10.1148/radiol.2412051122

subject

Has Abstract

pub_date

2006-11-01 00:00:00

pages

492-500

issue

eissn

0033-8419

issn

1527-1315

pii

241/2/492

journal_volume

241

pub_type

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Subcortical damage and cortical functional changes in men and women with Fabry disease: a multifaceted MR study.