학술논문

Prosaccade and Antisaccade Behavior in Fragile X‐Associated Tremor/Ataxia Syndrome Progression
Document Type
article
Source
Movement Disorders Clinical Practice. 9(4)
Subject
Biomedical and Clinical Sciences
Neurosciences
Clinical Sciences
Rare Diseases
Fragile X Syndrome
Neurodegenerative
Clinical Research
Brain Disorders
Intellectual and Developmental Disabilities (IDD)
FXTAS
prosaccade
antisaccade
eye movements
inhibitory deficits
inhibitory deficits.
Clinical sciences
Language
Abstract
BackgroundQuantitative measurement of eye movements can reveal subtle progression in neurodegenerative diseases.ObjectiveTo determine if quantitative measurements of eye movements may reveal subtle progression of fragile X-associated tremor and ataxia (FXTAS).MethodsProsaccade (PS) and antisaccade (AS) behavior was analyzed in 25 controls, 57 non-FXTAS carriers, and 46 carriers with FXTAS.ResultsSymptomatic individuals with FXTAS had longer AS latencies, increased rates of AS errors, and increased AS dysmetria relative to non-FXTAS carriers and controls. These deficits, along with PS latency and velocity, were greater in advanced FXTAS stages.ConclusionAS deficits differentiated FXTAS from non-FXTAS premutation carriers implicating top-down control and frontostriatal deterioration. However, the absence of group differences between non-FXTAS carriers and controls in AS and PS markers suggests saccade performance may not be a sensitive enough measure for detecting conversion to FXTAS, but instead more helpful as translational biomarkers of FXTAS progression.