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The pattern of cognitive performance in CADASIL: a monogenic condition leading to subcortical ischemic vascular

Nils Peters1, Christian Opherk, Adrian Danek

  • 1Department of Neurology, Klinikum Grosshadern, Ludwig-Maximilians-University, Marchioninistrasse 15, D-81377 Munich, Germany. mdichgans@nefo.med.uni-muenchen.de.

The American Journal of Psychiatry
|November 3, 2005
PubMed

Insights

Cognitive impairment in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) primarily affects processing speed and executive functions. These deficits are evident early, aiding targeted clinical trial development for small vessel disease.

Area of Science:

  • Neurology
  • Neuroscience
  • Genetics

Background:

  • Subcortical ischemic vascular lesions are linked to cognitive decline and dementia.
  • Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a genetic small vessel disease caused by NOTCH3 mutations.
  • CADASIL invariably leads to cognitive deficits and dementia in mutation carriers.

Purpose of the Study:

  • To characterize the specific cognitive abnormalities in individuals with CADASIL.
  • To identify the core cognitive profile associated with CADASIL and small vessel disease.

Main Methods:

  • A cross-sectional study involving 65 NOTCH3 mutation carriers and 30 matched controls.
  • Cognitive assessments included global cognition, executive function, attention, processing speed, and error monitoring.
  • Utilized tests such as the Vascular Dementia Assessment Scale, Stroop, Trail Making Test, and maze task.

Main Results:

  • CADASIL subjects showed significant impairments in timed measures, executive functions (verbal fluency, ideational praxis), and attention.
  • Processing speed was the most affected cognitive domain.
  • Error monitoring was also impaired, though to a lesser extent than processing speed. Recall, orientation, and receptive language were preserved.

Conclusions:

  • Processing speed is the primary cognitive deficit in CADASIL, with secondary impacts on executive performance and attention.
  • This cognitive profile is present early in the disease course.
  • The identified cognitive signature may represent the central cognitive syndrome of small vessel disease and subcortical ischemic lesions, valuable for clinical trial design.
Abstract

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