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Diagnostic utility of different MRI and MR angiography measures in Fabry disease

Fellgiebel, A. and Keller, I. and Marin, D. and Müller, M. J. and Schermuly, I. and Yakushev, I. and Albrecht, J. and Bellhäuser, Henrik and Kinateder, M. and Beck, M. and Stoeter, P. (2009):
Diagnostic utility of different MRI and MR angiography measures in Fabry disease.
In: Neurology, pp. 63-68, 72, (1), [Article]

Abstract

Background: Neurologic hallmarks of Fabry disease (FD) include small fiber neuropathy as well as cerebral micro- and macroangiopathy with premature stroke. Cranial MRI shows progressive white matter lesions (WML) at an early age, increased signal intensity in the pulvinar, and tortuosity and dilatation of the larger vessels. To unravel the most promising imaging tool for the detection of CNS involvement in FD we compared the diagnostic utility of the different MR imaging findings.

Methods: Twenty-five clinically affected patients with FD (age 36.5 ± 11.0) and 20 age-matched controls were investigated by structural MRI, MR angiography, and diffusion tensor imaging (DTI). Individual WML volumes, global mean diffusivity (MD), and mean cerebral artery diameters were determined.

Results: Using receiver operating characteristic analyses, enlarged diameters of the following cerebral arteries significantly separated patients with FD from controls: middle cerebral artery: area under curve (AUC) = 0.75, p = 0.005; posterior cerebral artery: AUC = 0.69, p = 0.041; carotid artery: 0.69, p = 0.041; basilar artery: AUC = 0.96, p < 0.0005. A total of 87% of the individuals were correctly classified by basilar artery diameters (sensitivity 95%, specificity 83%). WML volumes and global MD values did not significantly separate patients from controls.

Conclusions: With an accuracy of 87%, basilar artery diameters were superior to all other MR measures for separating patients with Fabry disease (FD) from controls. Future studies should adopt basilar artery measurements for early detection and monitoring of brain involvement in FD. Moreover, further investigations should reveal if the dilated vasculopathy in FD could be a screening marker to detect FD in a cohort of other cerebrovascular diseases, especially in cryptogenic stroke.

Item Type: Article
Erschienen: 2009
Creators: Fellgiebel, A. and Keller, I. and Marin, D. and Müller, M. J. and Schermuly, I. and Yakushev, I. and Albrecht, J. and Bellhäuser, Henrik and Kinateder, M. and Beck, M. and Stoeter, P.
Title: Diagnostic utility of different MRI and MR angiography measures in Fabry disease
Language: English
Abstract:

Background: Neurologic hallmarks of Fabry disease (FD) include small fiber neuropathy as well as cerebral micro- and macroangiopathy with premature stroke. Cranial MRI shows progressive white matter lesions (WML) at an early age, increased signal intensity in the pulvinar, and tortuosity and dilatation of the larger vessels. To unravel the most promising imaging tool for the detection of CNS involvement in FD we compared the diagnostic utility of the different MR imaging findings.

Methods: Twenty-five clinically affected patients with FD (age 36.5 ± 11.0) and 20 age-matched controls were investigated by structural MRI, MR angiography, and diffusion tensor imaging (DTI). Individual WML volumes, global mean diffusivity (MD), and mean cerebral artery diameters were determined.

Results: Using receiver operating characteristic analyses, enlarged diameters of the following cerebral arteries significantly separated patients with FD from controls: middle cerebral artery: area under curve (AUC) = 0.75, p = 0.005; posterior cerebral artery: AUC = 0.69, p = 0.041; carotid artery: 0.69, p = 0.041; basilar artery: AUC = 0.96, p < 0.0005. A total of 87% of the individuals were correctly classified by basilar artery diameters (sensitivity 95%, specificity 83%). WML volumes and global MD values did not significantly separate patients from controls.

Conclusions: With an accuracy of 87%, basilar artery diameters were superior to all other MR measures for separating patients with Fabry disease (FD) from controls. Future studies should adopt basilar artery measurements for early detection and monitoring of brain involvement in FD. Moreover, further investigations should reveal if the dilated vasculopathy in FD could be a screening marker to detect FD in a cohort of other cerebrovascular diseases, especially in cryptogenic stroke.

Journal or Publication Title: Neurology
Volume: 72
Number: 1
Divisions: 03 Department of Human Sciences > Institute for Psychology
03 Department of Human Sciences
Date Deposited: 23 Oct 2012 14:02
Additional Information:

Impact Factor: 8.17

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