Abstract

Isolated dystonia manifests with involuntary muscle hyperactivity, but the extent of cognitive impairment remains controversial. We examined the executive functions in blepharospasm while accounting for motor symptom-related distractions as a factor often limiting the interpretability of neuropsychological studies in dystonia. Our control group comprised of patients with hemifacial spasm, which is a condition producing similar motor symptoms without any central nervous system pathology. Nineteen patients with blepharospasm and 22 patients with hemifacial spasm completed a flanker task. Stimulus congruency on the current trial, on the preceding trial, and a response sequence served as independent variables. We analyzed the response time and accuracy. Gross overall group differences were not discernible. While congruency, congruency sequence, and response sequence exerted the expected effects, no group differences emerged with regard to these variables. A difference between patients with blepharospasm and those with hemifacial spasm consisted in longer reaction times when responses had to be repeated following stimulus incongruency on the preceding trial. We conclude that patients with blepharospasm seem to have difficulties in repeating their responses when incongruency on preceding trials interferes with habit formation or other forms of fast routes to action. Our specific finding may provide an opportunity to study altered basal ganglia plasticity in focal dystonia.

Highlights

  • Isolated dystonia is a neurological condition of impaired motor control resulting in involuntary muscle contractions, twisting movements, and sustained or intermittent abnormal postures [1]

  • We examined an important facet of executive function, i.e., response inhibition, as well as stimulus and response sequence effects in patients with BSP by means of an objective behavioral

  • We expected that BSP patients might perform worse than hemifacial spasm (HFS) patients on the flanker task, as many studies showed structural and functional abnormalities in basal ganglia (BG) and BG-related loops in focal dystonia [14,21,22,27], as well as cognitive and psychiatric disturbances in patients with focal dystonia [2,3,4,5,6,7,8]

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Summary

Introduction

Isolated dystonia is a neurological condition of impaired motor control resulting in involuntary muscle contractions, twisting movements, and sustained or intermittent abnormal postures [1]. There is emerging evidence that adult onset focal dystonia is not limited to motor impairment but may affect cognitive functions [2,3,4,5,6,7,8]. Other studies did not report cognitive impairments in patients with focal dystonia [9,10,11]. Structural and/or functional changes in focal dystonia may be present in the basal ganglia (BG). The BG hypothesis of focal dystonia was derived from studies focusing on these subcortical

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