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

Publikationstyp:
Zeitschriftenaufsatz
Metadaten:
Autoren
  • A Fellgiebel
  • I Keller
  • D Marin
  • MJ Mueller
  • I Schermuly
  • I Yakushev
  • J Albrecht
  • H Bellhaeuser
  • M Kinateder
  • M Beck
  • P Stoeter
Autoren-URL
https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=fis-test-1&SrcAuth=WosAPI&KeyUT=WOS:000262162600011&DestLinkType=FullRecord&DestApp=WOS_CPL
DOI
10.1212/01.wnl.0000338566.54190.8a
Externe Identifier
  • Clarivate Analytics Document Solution ID: 390KL
  • PubMed Identifier: 19122032
ISSN
0028-3878
Ausgabe der Veröffentlichung
1
Zeitschrift
NEUROLOGY
Paginierung
63 - 68
Datum der Veröffentlichung
2009
Status
Published
Titel
Diagnostic utility of different MRI and MR angiography measures in Fabry disease
Sub types
  • Article
Ausgabe der Zeitschrift
72

Datenquelle: Web of Science (Lite)

Andere Metadatenquellen:
Autoren
  • A Fellgiebel
  • I Keller
  • D Marin
  • MJ Müller
  • I Schermuly
  • I Yakushev
  • J Albrecht
  • H Bellhäuser
  • M Kinateder
  • M Beck
  • P Stoeter
DOI
10.1212/01.wnl.0000338566.54190.8a
eISSN
1526-632X
ISSN
0028-3878
Ausgabe der Veröffentlichung
1
Zeitschrift
Neurology
Sprache
en
Paginierung
63 - 68
Datum der Veröffentlichung
2009
Status
Published
Herausgeber
Ovid Technologies (Wolters Kluwer Health)
Herausgeber URL
http://dx.doi.org/10.1212/01.wnl.0000338566.54190.8a
Datum der Datenerfassung
2024
Titel
Diagnostic utility of different MRI and MR angiography measures in Fabry disease
Ausgabe der Zeitschrift
72

Datenquelle: Crossref

Abstract
<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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.<h4>Conclusions</h4>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.
Addresses
  • Department of Psychiatry, University Hospital of Mainz, Untere Zahlbacher Str. 8, 55131 Mainz, Germany. fellgiebel@psychiatrie.klinik.uni-mainz.de
Autoren
  • A Fellgiebel
  • I Keller
  • D Marin
  • MJ Müller
  • I Schermuly
  • I Yakushev
  • J Albrecht
  • H Bellhäuser
  • M Kinateder
  • M Beck
  • P Stoeter
DOI
10.1212/01.wnl.0000338566.54190.8a
eISSN
1526-632X
Externe Identifier
  • PubMed Identifier: 19122032
Open access
false
ISSN
0028-3878
Ausgabe der Veröffentlichung
1
Zeitschrift
Neurology
Schlüsselwörter
  • Cerebral Arteries
  • Brain
  • Humans
  • Fabry Disease
  • Diagnosis, Differential
  • Imaging, Three-Dimensional
  • Magnetic Resonance Imaging
  • Diffusion Magnetic Resonance Imaging
  • Cerebral Angiography
  • Brain Mapping
  • Area Under Curve
  • Sensitivity and Specificity
  • Case-Control Studies
  • Adult
  • Middle Aged
  • Female
  • Male
Sprache
eng
Medium
Print
Paginierung
63 - 68
Datum der Veröffentlichung
2009
Status
Published
Datum der Datenerfassung
2009
Titel
Diagnostic utility of different MRI and MR angiography measures in Fabry disease.
Sub types
  • Comparative Study
  • Journal Article
Ausgabe der Zeitschrift
72

Datenquelle: Europe PubMed Central

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.
Autoren
  • A Fellgiebel
  • I Keller
  • D Marin
  • MJ Müller
  • I Schermuly
  • I Yakushev
  • J Albrecht
  • H Bellhäuser
  • M Kinateder
  • M Beck
  • P Stoeter
Autoren-URL
https://www.ncbi.nlm.nih.gov/pubmed/19122032
DOI
10.1212/01.wnl.0000338566.54190.8a
eISSN
1526-632X
Ausgabe der Veröffentlichung
1
Zeitschrift
Neurology
Schlüsselwörter
  • Adult
  • Area Under Curve
  • Brain
  • Brain Mapping
  • Case-Control Studies
  • Cerebral Angiography
  • Cerebral Arteries
  • Diagnosis, Differential
  • Diffusion Magnetic Resonance Imaging
  • Fabry Disease
  • Female
  • Humans
  • Imaging, Three-Dimensional
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Sensitivity and Specificity
Sprache
eng
Country
United States
Paginierung
63 - 68
PII
72/1/63
Datum der Veröffentlichung
2009
Status
Published
Datum, an dem der Datensatz öffentlich gemacht wurde
2009
Titel
Diagnostic utility of different MRI and MR angiography measures in Fabry disease.
Sub types
  • Comparative Study
  • Journal Article
Ausgabe der Zeitschrift
72

Datenquelle: PubMed

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