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Related Experiment Videos

Cognitive alterations in non-demented CADASIL patients

H Taillia1, H Chabriat, A Kurtz

  • 1Service de Neurologie, Hôpital Saint-Antoine, Necker, Paris, France.

Cerebrovascular Diseases (Basel, Switzerland)
|April 21, 1998
PubMed
Summary

Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) patients show subtle cognitive impairment, particularly affecting frontal lobe functions, even before dementia develops. This deficit is not linked to MRI-visible brain lesion severity.

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Area of Science:

  • Neurology
  • Neurogenetics
  • Cognitive Neuroscience

Background:

  • Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is an inherited small-artery brain disease caused by Notch3 gene mutations.
  • CADASIL is characterized by strokes, migraine with aura, mood disturbances, and progressive subcortical dementia.
  • The exact timing of cognitive decline onset in CADASIL is not well-established.

Purpose of the Study:

  • To evaluate cognitive functions in symptomatic, non-demented patients with CADASIL.
  • To identify specific cognitive deficits and their relationship to disease progression and brain imaging findings.

Main Methods:

  • Cognitive assessment of 8 symptomatic, non-demented CADASIL patients (aged 35-66).
  • Utilized tests including Wisconsin Card-Sorting Test (WCST), Trail-Making Test, Rey's figure copying, WAIS-R, Wechsler Memory Scale, Raven's Progressive Matrices, and fluency tasks.

Related Experiment Videos

  • Correlated WCST scores with MRI findings of white matter and basal ganglia abnormalities.

    • Main Results:

    • All patients exhibited altered performance on the WCST, indicating executive function deficits.
    • Impaired performance was also noted on the Trail-Making Test (5/8) and Rey's figure copying (3/8).
    • Subtle cognitive impairment, particularly affecting frontal lobe tasks, is present in non-demented CADASIL patients and is not strongly correlated with MRI lesion severity.

    Conclusions:

    • Symptomatic CADASIL patients, even without dementia, can exhibit subtle cognitive impairments.
    • Executive functions, assessed by tasks involving the frontal lobes, are most frequently affected.
    • These cognitive deficits can emerge independently of major vascular events and do not correlate with the extent of visible brain lesions on MRI.