Journal article
Subcortical volumetric reductions in adult niemann-pick disease type C: A cross-sectional study
M Walterfang, B Patenaude, LA Abel, H Kluenemann, EA Bowman, MC Fahey, P Desmond, W Kelso, D Velakoulis
American Journal of Neuroradiology | Published : 2013
DOI: 10.3174/ajnr.A3356
Abstract
BACKGROUND AND PURPOSE: Voxel-based analysis has suggested that deep gray matter rather than cortical regions is initially affected in adult Niemann-Pick type C. We sought to examine a range of deep gray matter structures in adults with NPC and relate these to clinical variables. MATERIALS AND METHODS: Ten adult patients with NPC (18-49 years of age) were compared with 27 age- and sex-matched controls, and subcortical structures were automatically segmented from normalized T1-weighted MR images. Absolute volumes (in cubic millimeters) were generated for a range of deep gray matter structures and were compared between groups and correlated with illness variables. RESULTS: Most structures were..
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Funding Acknowledgements
Mark Walterfang-UNRELATED: Consultancy: Actelion Pharmaceuticals, Comments: International advisory board, Payment for Lectures (including service on Speakers Bureaus): Actelion, Comments: payment of honorarium for speaking at International NPC forum, Royalties: Australian Council for Educational Research, Comments: payment of royalties for the NUCOG, a pen-and-paper cognitive screening tool. Brian Patenaude-OTHER RELATIONSHIPS: I work with Oxford University (the FSL group) in development of the subcortical segmentation tool. No compensation for these activities is received. Larry A. Abel-UNRELATED: Consultancy: Actelion,<SUP>star</SUP> Comments: consulting fee for Miglustat clinical trials (2004-2005), Payment for Lectures (including service on Speakers Bureaus): Actelion,<SUP>star</SUP> Comments: honoraria for presentations at 2 Actelion-sponsored meetings on NPC in Athens, Greece, March 2012 and Prague, Czech Republic, June 2012. Hans Klunemann-UNRELATED: Board Membership: Actelion, Consultancy: Actelion, Grants/Grants Pending: Actelion;<SUP>star</SUP> Payment for Lectures (including service on Speakers Bureaus): Actelion, Comments: lectures about dementia for Pfizer, Lilly, GlaxoSmithKline, Actelion, Lundbeck, Merz Pharmaceuticals, Payment for Development of Educational Presentations: Actelion, Travel/Accommodations/Meeting Expenses Unrelated to Activities Listed: Actelion. Michael Fahey-UNRELATED: Board Membership, Consultancy: Actelion, Comments: honoraria for advisory board membership and consultancy. Patricia Desmond-UNRELATED: Grants/Grants Pending: National Health and Medical Research Council of Australia,<SUP>star</SUP> Comments: awarded to an affiliated institution of Melbourne University for a different research project. Dennis Velakoulis-UNRELATED: Royalties: Royalties received for the cognitive screening tool NUCOG. <SUP>star</SUP>Money paid to the institution.