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Related Experiment Videos

Insidious cognitive decline in CADASIL.

Kaarina Amberla1, Minna Wäljas, Susanna Tuominen

  • 1Division of Clinical Geriatrics, Karolinska Institutet, Stockholm, Sweden.

Stroke
|May 15, 2004
PubMed
Summary

Cognitive decline in CADASIL, a genetic condition, begins before stroke, impacting working memory and executive function. Early detection through specific tests can distinguish carriers from healthy individuals.

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Area of Science:

  • Neurology
  • Genetics
  • Cognitive Science

Background:

  • Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetic condition.
  • CADASIL leads to recurrent ischemic attacks and subcortical vascular dementia.

Purpose of the Study:

  • To characterize cognitive function in individuals with the C475T (R133C) Notch3 gene mutation causing CADASIL.
  • To identify early cognitive changes associated with CADASIL.

Main Methods:

  • Compared prestroke and poststroke mutation carriers with dementia and healthy non-carriers.
  • Utilized a comprehensive neuropsychological test battery.

Main Results:

  • Working memory and executive function deficits were evident pre-stroke.

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  • Post-stroke, impairments extended to mental speed and visuospatial ability.
  • Three tests reliably distinguished carriers from controls, assessing working memory/attention, executive function, and mental speed.
  • Conclusions:

    • Cognitive decline in CADASIL initiates insidiously in the pre-stroke phase.
    • Working memory and executive function deterioration precede symptomatic ischemic episodes.