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Dementia is an acquired, progressive syndrome characterized by a decline in multiple cognitive domains severe enough to impair daily functioning and reduce independence. Although memory loss is a central feature, the diagnosis requires additional deficits involving language, executive function, visuospatial skills, judgment, calculation, or abstract reasoning. These cognitive impairments reflect underlying neurodegenerative or vascular processes that gradually disrupt neuronal networks...
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Related Experiment Video

Updated: May 10, 2026

A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia
07:33

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Published on: May 21, 2010

Progressive cognitive dysfunction in spinocerebellar ataxia type 3.

Sandra Roeske1, Ina Filla, Stefan Heim

  • 1German Center for Neurodegenerative Diseases, Bonn, Germany; Department of Neurology, University Hospital of Bonn, Bonn, Germany.

Movement Disorders : Official Journal of the Movement Disorder Society
|June 6, 2013
PubMed
Summary

Cognitive dysfunction in spinocerebellar ataxia type 3 (SCA3) worsens over time. Memory and learning abilities significantly declined, indicating cognitive decline is integral to SCA3 progression.

Keywords:
SCA3ataxiacerebellumcognitionfollow-up

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08:51

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Published on: December 5, 2020

Area of Science:

  • Neuroscience
  • Genetics
  • Neurology

Background:

  • Cognitive dysfunction is known in spinocerebellar ataxia type 3 (SCA3).
  • The progression of cognitive impairment in SCA3 has not been previously studied.

Purpose of the Study:

  • To prospectively investigate the progression of cognitive function in patients with SCA3.

Main Methods:

  • Eleven SCA3 patients underwent a comprehensive neuropsychological test battery.
  • Patients were retested after an average of 3.5 years to assess cognitive changes.

Main Results:

  • Significant deterioration was observed in verbal learning, verbal memory, and figural memory.
  • Motor control and ataxia also worsened, but no increase in depressive symptoms was noted.

Conclusions:

  • Cognitive decline, particularly in memory and learning, is an integral part of SCA3 progression.
  • This decline is not solely due to motor impairment, suggesting extracerebellar pathology or disrupted cerebellar-cerebral circuits.