Prepulse Inhibition in Patients with Fragile X-associated Tremor Ataxia Syndrome

Overview

Journal Neurobiol Aging

Publisher Elsevier

Specialties Geriatrics
Neurology
Physiology

Date 2010 Oct 22

PMID 20961665

Citations 14

Authors

Andrea Schneider

Elizabeth Ballinger

Alyssa Chavez

Flora Tassone

Randi J Hagerman

David Hessl

Affiliations

Soon will be listed here.

Abstract

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.

Citing Articles

Nigrostriatal Degeneration Underpins Sensorimotor Dysfunction in an Inducible Mouse Model of Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS).

Kul E, Santos M, Stork O Int J Mol Sci. 2025; 26(4).

PMID: 40003975 PMC: 11855849. DOI: 10.3390/ijms26041511.

Case Series: Vestibular Migraines in Fragile X Premutation Carriers.

Tak Y, Tassone F, Hagerman R J Clin Med. 2024; 13(2).

PMID: 38256638 PMC: 10816080. DOI: 10.3390/jcm13020504.

Cognitive Dysfunction in Repeat Expansion Diseases: A Review.

Zhang S, Shen L, Jiao B Front Aging Neurosci. 2022; 14:841711.

PMID: 35478698 PMC: 9036481. DOI: 10.3389/fnagi.2022.841711.

Precision Sensorimotor Control in Aging FMR1 Gene Premutation Carriers.

McKinney W, Wang Z, Kelly S, Khemani P, Lui S, White S Front Integr Neurosci. 2019; 13:56.

PMID: 31632248 PMC: 6783559. DOI: 10.3389/fnint.2019.00056.

What has been learned from mouse models of the Fragile X Premutation and Fragile X-associated tremor/ataxia syndrome?.

Foote M, Careaga M, Berman R Clin Neuropsychol. 2016; 30(6):960-72.

PMID: 27355912 PMC: 5370556. DOI: 10.1080/13854046.2016.1158254.

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