Interaction between ventricular expansion and structural changes in the corpus callosum and putamen in males with FMR1 normal and premutation alleles

NEUROBIOLOGY OF AGING

卷:86

Article

Wang, Jun Yi^1,2,3  Hessl, David^2,4  Tassone, Flora^2,3  Kim, Kyoungmi^2,5  Hagerman, Randi J.^2,6  Rivera, Susan M.^1,2,7 

[1] Univ Calif Davis, Ctr Mind & Brain, 267 Cousteau Pl, Davis, CA 95618 USA

[2] Univ Calif Davis, Med Ctr, MIND Inst, Sacramento, CA 95817 USA

[3] Univ Calif Davis, Sch Med, Dept Biochem & Mol Med, Sacramento, CA 95817 USA

[4] Univ Calif Davis, Sch Med, Dept Psychiat & Behav Sci, Sacramento, CA 95817 USA

[5] Univ Calif Davis, Sch Med, Dept Publ Hlth Sci, Sacramento, CA 95817 USA

[6] Univ Calif Davis, Sch Med, Dept Pediat, Sacramento, CA 95817 USA

[7] Univ Calif Davis, Dept Psychol, Davis, CA 95618 USA

关键词: Fragile X premutation;    FXTAS;    FMR1;    Normal pressure hydrocephalus;    MRI;    Neurodegenerative disorder;   

DOI  :  10.1016/j.neurobiolaging.2019.09.009

来源: Elsevier

【 摘 要 】

Ventricular enlargement (VE) is commonly observed in aging and fragile X-associated tremor/ataxia syndrome (FXTAS), a late-onset neurodegenerative disorder. VE may generate a mechanical force causing structural deformation. In this longitudinal study, we examined the relationships between VE and structural changes in the corpus callosum (CC) and putamen. MRI scans (2-7/person over 0.2-7.5 years) were acquired from 22 healthy controls, 26 unaffected premutation carriers (PFX-), and 39 carriers affected with FXTAS (PFX+). Compared with controls, PFX- demonstrated enlarged fourth ventricles, whereas PFX+ displayed enlargement in both third and fourth ventricles, CC thinning, putamen atrophy/ deformation (thinning and increased distance), and accelerated expansions in lateral ventricles. Common for all groups, baseline VE predicted accelerated CC thinning and putamen atrophy/deformation and conversely, baseline CC and putamen atrophy/deformation and enlarged third and fourth ventricles predicted accelerated lateral ventricular expansion. The results suggest a progressive VE within the 4 ventricles as FXTAS develops and a deleterious cycle between VE and brain deformation that may commonly occur during aging and FXTAS progression but become accelerated in FXTAS. (C) 2019 Elsevier Inc. All rights reserved.

【 授权许可】

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