Selective vulnerability to atrophy in sporadic Creutzfeldt-Jakob disease
Younes, Kyan (University of California, San Francisco (UCSF))
Rojas, Julio C. (University of California, San Francisco (UCSF))
Wolf, Amy (University of California, San Francisco (UCSF))
Sheng-Yang, Goh M. (University of California, San Francisco (UCSF))
Paoletti, Matteo (IRCCS Mondino Foundation)
Toller, Gianina (University of California, San Francisco (UCSF))
Caverzasi, Eduardo (University of California, San Francisco (UCSF))
Mandelli, Maria Luisa (University of California, San Francisco (UCSF))
Illán-Gala, Ignacio (Institut d'Investigació Biomèdica Sant Pau)
Kramer, Joel H. (University of California, San Francisco (UCSF))
Cobigo, Yann (University of California, San Francisco (UCSF))
Miller, Bruce L. (University of California, San Francisco (UCSF))
Rosen, Howard J. (University of California, San Francisco (UCSF))
Geschwind, Michael D. (University of California, San Francisco (UCSF))
Universitat Autònoma de Barcelona

Data:	2021
Resum:	Identification of brain regions susceptible to quantifiable atrophy in sporadic Creutzfeldt-Jakob disease (sCJD) should allow for improved understanding of disease pathophysiology and development of structural biomarkers that might be useful in future treatment trials. Although brain atrophy is not usually present by visual assessment of MRIs in sCJD, we assessed whether using voxel-based morphometry (VBM) can detect group-wise brain atrophy in sCJD. 3T brain MRI data were analyzed with VBM in 22 sCJD participants and 26 age-matched controls. Analyses included relationships of regional brain volumes with major clinical variables and dichotomization of the cohort according to expected disease duration based on prion molecular classification (i. e. , short-duration/Fast-progressors (MM1, MV1, and VV2) vs. long-duration/Slow-progressors (MV2, VV1, and MM2)). Structural equation modeling (SEM) was used to assess network-level interactions of atrophy between specific brain regions. sCJD showed selective atrophy in cortical and subcortical regions overlapping with all but one region of the default mode network (DMN) and the insulae, thalami, and right occipital lobe. SEM showed that the effective connectivity model fit in sCJD but not controls. The presence of visual hallucinations correlated with right fusiform, bilateral thalami, and medial orbitofrontal atrophy. Interestingly, brain atrophy was present in both Fast- and Slow-progressors. Worse cognition was associated with bilateral mesial frontal, insular, temporal pole, thalamus, and cerebellum atrophy. Brain atrophy in sCJD preferentially affects specific cortical and subcortical regions, with an effective connectivity model showing strength and directionality between regions. Brain atrophy is present in Fast- and Slow-progressors, correlates with clinical findings, and is a potential biomarker in sCJD.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, i la comunicació pública de l'obra, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original. No es permet la creació d'obres derivades.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Publicat a:	Annals of Clinical and Translational Neurology, Vol. 8 (may 2021) , p. 1183-1199, ISSN 2328-9503

DOI: 10.1002/acn3.51290
PMID: 33949799

17 p, 1023.4 KB

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