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Imaging of neuroinflammation in adult Niemann-Pick type C disease: A cross-sectional study.

Authors :
Walterfang M
Di Biase MA
Cropley VL
Scott AM
O'Keefe G
Velakoulis D
Pathmaraj K
Ackermann U
Pantelis C
Source :
Neurology [Neurology] 2020 Apr 21; Vol. 94 (16), pp. e1716-e1725. Date of Electronic Publication: 2020 Mar 24.
Publication Year :
2020

Abstract

Objective: To test the hypothesis that neuroinflammation is a key process in adult Niemann-Pick type C (NPC) disease, we undertook PET scanning utilizing a ligand binding activated microglia on 9 patients and 9 age- and sex-matched controls.<br />Method: We scanned all participants with the PET radioligand <superscript>11</superscript> C-(R)-PK-11195 and undertook structural MRI to measure gray matter volume and white matter fractional anisotropy (FA).<br />Results: We found increased binding of <superscript>11</superscript> C-(R)-PK-11195 in total white matter compared to controls ( p < 0.01), but not in gray matter regions, and this did not correlate with illness severity or duration. Gray matter was reduced in the thalamus ( p < 0.0001) in patients, who also showed widespread reductions in FA across the brain compared to controls ( p < 0.001). A significant correlation between <superscript>11</superscript> C-(R)-PK11195 binding and FA was shown ( p = 0.002), driven by the NPC patient group.<br />Conclusions: Our findings suggest that neuroinflammation-particularly in white matter-may underpin some structural and degenerative changes in patients with NPC.<br /> (© 2020 American Academy of Neurology.)

Details

Language :
English
ISSN :
1526-632X
Volume :
94
Issue :
16
Database :
MEDLINE
Journal :
Neurology
Publication Type :
Academic Journal
Accession number :
32209649
Full Text :
https://doi.org/10.1212/WNL.0000000000009287