Journal article

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

Mark Walterfang, Maria A Di Biase, Vanessa L Cropley, Andrew M Scott, Graeme O'Keefe, Dennis Velakoulis, Kunthi Pathmaraj, Uwe Ackermann, Christos Pantelis

Neurology | American Academy of Neurology | Published : 2020


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. Method: We scanned all participants with the PET radioligand 11C-(R)-PK-11195 and undertook structural MRI to measure gray matter volume and white matter fractional anisotropy (FA). Results: We found increased binding of 11C-(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 patient..

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