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Disease Severity Staging System for NOTCH3-Associated Small Vessel Disease, Including CADASIL.

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A new staging system for NOTCH3-related small vessel disease (SVD) effectively categorizes disease severity from premanifest to end-stage. This validated system aids in clinical and research settings for understanding NOTCH3-SVD progression.

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Area of Science:

  • Neurology
  • Genetics
  • Vascular Medicine

Background:

  • Cysteine-altering NOTCH3 (NOTCH3cys) variants are common, affecting ~1 in 300 individuals.
  • These variants are linked to a wide range of small vessel disease (SVD) severities, including cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), stroke, dementia, and asymptomatic cases.
  • A standardized system to classify NOTCH3-SVD severity is currently lacking.

Purpose of the Study:

  • To develop a straightforward staging system for disease severity that encompasses the full clinicoradiological spectrum of NOTCH3-SVD.
  • To validate this staging system in independent cohorts.

Main Methods:

  • A cohort study was conducted, developing the NOTCH3-SVD staging system in a discovery cohort and validating it in international CADASIL cohorts and the UK Biobank.
  • Data from 2,000+ participants with NOTCH3cys variants, including brain MRI and clinical assessments, were analyzed.
  • Follow-up data spanning up to 18 years were incorporated.

Main Results:

  • The developed NOTCH3-SVD staging system includes 9 stages (0 to 4B), capturing the spectrum from premanifest to end-stage disease.
  • Ninety-four percent of participants (1789/1908) followed the defined sequence of disease events.
  • The stages correlated significantly with neuroimaging findings, cognitive function, serum neurofilament light chain levels, and 18-year survival.

Conclusions:

  • The NOTCH3-SVD staging system effectively captures the complete disease spectrum in individuals with NOTCH3cys variants.
  • This system provides a simple, validated tool for consistent disease staging in both clinical practice and research.
  • It aids in understanding disease progression and outcomes associated with NOTCH3-SVD.