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Imaging of neuroinflammation in adult Niemann-Pick type C disease: A cross-sectional study.
Walterfang, Mark; Di Biase, Maria A; Cropley, Vanessa L; Scott, Andrew M; O'Keefe, Graeme; Velakoulis, Dennis; Pathmaraj, Kunthi; Ackermann, Uwe; Pantelis, Christos.
Affiliation
  • Walterfang M; From the Neuropsychiatry Unit (M.W., D.V.), Royal Melbourne Hospital; Melbourne Neuropsychiatry Centre (M.W., M.A.D., V.L.C., D.V., C.P.), The University of Melbourne & North Western Mental Health; The Florey Institute of Neuroscience and Mental Health (M.W., C.P.), Department of Psychiatry (M.W
  • Di Biase MA; From the Neuropsychiatry Unit (M.W., D.V.), Royal Melbourne Hospital; Melbourne Neuropsychiatry Centre (M.W., M.A.D., V.L.C., D.V., C.P.), The University of Melbourne & North Western Mental Health; The Florey Institute of Neuroscience and Mental Health (M.W., C.P.), Department of Psychiatry (M.W
  • Cropley VL; From the Neuropsychiatry Unit (M.W., D.V.), Royal Melbourne Hospital; Melbourne Neuropsychiatry Centre (M.W., M.A.D., V.L.C., D.V., C.P.), The University of Melbourne & North Western Mental Health; The Florey Institute of Neuroscience and Mental Health (M.W., C.P.), Department of Psychiatry (M.W
  • Scott AM; From the Neuropsychiatry Unit (M.W., D.V.), Royal Melbourne Hospital; Melbourne Neuropsychiatry Centre (M.W., M.A.D., V.L.C., D.V., C.P.), The University of Melbourne & North Western Mental Health; The Florey Institute of Neuroscience and Mental Health (M.W., C.P.), Department of Psychiatry (M.W
  • O'Keefe G; From the Neuropsychiatry Unit (M.W., D.V.), Royal Melbourne Hospital; Melbourne Neuropsychiatry Centre (M.W., M.A.D., V.L.C., D.V., C.P.), The University of Melbourne & North Western Mental Health; The Florey Institute of Neuroscience and Mental Health (M.W., C.P.), Department of Psychiatry (M.W
  • Velakoulis D; From the Neuropsychiatry Unit (M.W., D.V.), Royal Melbourne Hospital; Melbourne Neuropsychiatry Centre (M.W., M.A.D., V.L.C., D.V., C.P.), The University of Melbourne & North Western Mental Health; The Florey Institute of Neuroscience and Mental Health (M.W., C.P.), Department of Psychiatry (M.W
  • Pathmaraj K; From the Neuropsychiatry Unit (M.W., D.V.), Royal Melbourne Hospital; Melbourne Neuropsychiatry Centre (M.W., M.A.D., V.L.C., D.V., C.P.), The University of Melbourne & North Western Mental Health; The Florey Institute of Neuroscience and Mental Health (M.W., C.P.), Department of Psychiatry (M.W
  • Ackermann U; From the Neuropsychiatry Unit (M.W., D.V.), Royal Melbourne Hospital; Melbourne Neuropsychiatry Centre (M.W., M.A.D., V.L.C., D.V., C.P.), The University of Melbourne & North Western Mental Health; The Florey Institute of Neuroscience and Mental Health (M.W., C.P.), Department of Psychiatry (M.W
  • Pantelis C; From the Neuropsychiatry Unit (M.W., D.V.), Royal Melbourne Hospital; Melbourne Neuropsychiatry Centre (M.W., M.A.D., V.L.C., D.V., C.P.), The University of Melbourne & North Western Mental Health; The Florey Institute of Neuroscience and Mental Health (M.W., C.P.), Department of Psychiatry (M.W
Neurology ; 94(16): e1716-e1725, 2020 04 21.
Article in En | MEDLINE | ID: mdl-32209649
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 patients, who also showed widespread reductions in FA across the brain compared to controls (p < 0.001). A significant correlation between 11C-(R)-PK11195 binding and FA was shown (p = 0.002), driven by the NPC patient group. CONCLUSIONS: Our findings suggest that neuroinflammation-particularly in white matter-may underpin some structural and degenerative changes in patients with NPC.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Brain / Niemann-Pick Disease, Type C / Gray Matter / White Matter / Inflammation Type of study: Observational_studies / Prevalence_studies / Risk_factors_studies Limits: Adult / Female / Humans / Male Language: En Journal: Neurology Year: 2020 Document type: Article Country of publication:
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Brain / Niemann-Pick Disease, Type C / Gray Matter / White Matter / Inflammation Type of study: Observational_studies / Prevalence_studies / Risk_factors_studies Limits: Adult / Female / Humans / Male Language: En Journal: Neurology Year: 2020 Document type: Article Country of publication: