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3 results

  • Chapter 43 - Chorea

  • from Section 3: - Hyperkinetic Movement Disorders
  • Edited by Erik Ch. Wolters, Universität Zürich, Christian R. Baumann, Universität Zürich
  • Book:
    International Compendium of Movement Disorders
    Published online:
    07 January 2025
    Print publication:
    06 February 2025, pp 540-556

  • Summary

    Chorea is a state of excessive, spontaneous movements, irregularly timed, non-repetitive, randomly distributed, and abrupt in character. The severity of movements may vary from restlessness with mild intermittent exaggeration of gesture and expression, fidgeting movements of the hands, and dance-like gait to a continuous flow of disabling violent movements. The pathogenesis of chorea is mainly related to striatal dysfunction. The classification of the causes of choreatic dyskinesias is complex and involves many clinical entities, both hereditary and acquired. The severity of chorea can be reduced by antipsychotics, tetrabenazine or benzodiazepines.

  • Longitudinal Study of Cognitive Functioning in Friedreich’s Ataxia

  • Atteneri Hernández-Torres, Fernando Montón, Stephany Hess Medler, Érika de Nóbrega, Antonieta Nieto
  • Journal:
    Journal of the International Neuropsychological Society / Volume 27 / Issue 4 / April 2021
    Published online by Cambridge University Press:
    14 October 2020, pp. 343-350
  • Objective:

    Friedreich’s ataxia (FRDA) is the most common hereditary ataxia. It is a neurodegenerative disorder, characterized by progressive ataxia. FRDA is also associated with cognitive impairments. To date, the evolution of cognitive functioning is unknown. Our aim was to investigate the changes in the cognitive functioning of FRDA patients over an average eight-year timeframe. In addition, we aimed to study the relationship between cognitive changes and clinical variables.

    Methods:

    Twenty-nine FRDA patients who had been part of the sample of a previous study participated in the present study. The mean average time between the two assessments was 8.24 years. The participants completed an extensive battery of neuropsychological tests chosen to examine cognitive functioning in various cognitive domains: processing speed, attention, working memory, executive functions, verbal and visual memory, visuoperceptive and visuospatial skills, visuoconstructive functions and language.

    Results:

    At follow-up, cerebellar symptoms had worsened, and patients presented greater disability. Differences between baseline and follow-up were observed in motor and cognitive reaction times, several trials of the Stroop test, semantic fluency, and block designs. No other cognitive changes were observed. Deterioration in simple cognitive reactions times and block designs performance correlated with the progression of cerebellar symptoms.

    Conclusions:

    Our study has demonstrated for the first time that patients with FRDA experience a significant decline over time in several cognitive domains. Specifically, after an eight-year period, FRDA patients worsened in processing speed, fluency, and visuoconstructive skills. This progression is unlikely to be due to greater motor or speech impairment.