Document Detail


Prepulse inhibition in patients with fragile X-associated tremor ataxia syndrome.
MedLine Citation:
PMID:  20961665     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late onset neurodegenerative disorder that affects carriers of the fragile X premutation, typically after age 50. Common symptoms include intention tremor, ataxia, neuropathy, autonomic dysfunction, cognitive decline, and dementia. The objectives of this study were to determine if patients with FXTAS have altered prepulse inhibition (PPI; a measure of sensorimotor gating), and to study possible correlations between PPI, molecular status, and cognitive performance. A passive acoustic PPI paradigm was applied in 163 subjects; 121 carriers of the fragile X premutation, and 42 healthy controls. There were significant differences in PPI between premutation carriers with FXTAS and controls at PPI 60 ms, and at 120 ms. This effect was more prominent in the male FXTAS patients. There was a tendency to an impaired PPI in female premutation carriers at the 120 ms condition. There was a significant correlation between the PPI deficit and a higher CGG repeat number. The results show an impairment in sensorimotor gating processes in male carriers of the fragile X premutation, which is more prominent in patients with FXTAS.
Authors:
Andrea Schneider; Elizabeth Ballinger; Alyssa Chavez; Flora Tassone; Randi J Hagerman; David Hessl
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.     Date:  2010-10-20
Journal Detail:
Title:  Neurobiology of aging     Volume:  33     ISSN:  1558-1497     ISO Abbreviation:  Neurobiol. Aging     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-04-10     Completed Date:  2013-01-24     Revised Date:  2014-09-19    
Medline Journal Info:
Nlm Unique ID:  8100437     Medline TA:  Neurobiol Aging     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1045-53     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 Elsevier Inc. All rights reserved.
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MeSH Terms
Descriptor/Qualifier:
Acoustic Stimulation / methods
Aged
Ataxia / epidemiology,  genetics,  physiopathology*
Female
Fragile X Mental Retardation Protein / genetics
Fragile X Syndrome / epidemiology,  genetics,  physiopathology*
Humans
Male
Middle Aged
Neural Inhibition* / genetics
Startle Reaction / genetics
Tremor / epidemiology,  genetics,  physiopathology*
Grant Support
ID/Acronym/Agency:
90DD05969/DD/NCBDD CDC HHS; AG032115/AG/NIA NIH HHS; AG032119/AG/NIA NIH HHS; HD03671/HD/NICHD NIH HHS; K23 MH077554/MH/NIMH NIH HHS; K23 MH077554-01A1/MH/NIMH NIH HHS; K23 MH077554-05/MH/NIMH NIH HHS; MH77554/MH/NIMH NIH HHS; R01 HD036071/HD/NICHD NIH HHS; R01 HD036071-13/HD/NICHD NIH HHS; R01 MH078041/MH/NIMH NIH HHS; RL1 AG032115/AG/NIA NIH HHS; RL1 AG032115-05/AG/NIA NIH HHS; RL1 AG032119/AG/NIA NIH HHS; RL1 AG032119-05/AG/NIA NIH HHS; UL1 DE019583/DE/NIDCR NIH HHS; UL1 DE019583-05/DE/NIDCR NIH HHS; UL1 RR024146/RR/NCRR NIH HHS; UL1 RR024146/RR/NCRR NIH HHS; UL1 RR024146-01/RR/NCRR NIH HHS; UL1 RR024146-05/RR/NCRR NIH HHS; UL1DE019583/DE/NIDCR NIH HHS
Chemical
Reg. No./Substance:
0/FMR1 protein, human; 139135-51-6/Fragile X Mental Retardation Protein
Comments/Corrections

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