A tool to determine disease stages

NOTCH3-SVD staging system

The NOTCH3-SVD severity staging system offers a simple and standardized approach to assigning individuals with a CADASIL-type NOTCH3 variant into different disease stages, from pre-manifest to end-stage disease. This online tool allows for assessment of a single case and multiple cases simultaneously.

NOTCH3-SVD staging: single case

NOTCH3-SVD staging: multiple cases

Paste your cohort data in csv-format below. Use the exact column headers: ‘CaseID,Fazekas,Lacune_count,mRS’ and use a new line for each case. Click 'Calculate Stages’ to process the data. An output csv-file will be downloaded automatically with a list the cases and their NOTCH3-SVD stages. The summary statistics of your cohort will be shown below.

Please note that this tool only works for complete data (i.e. a known Fazekas score, Lacune count and mRS score for each case).




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Warning: {{ error.message }} in row {{ error.row + 1 }}

Loaded data

{{ key }}
{{ (index == "N3SVD-stage") ? stageNames[cellValue] : cellValue }}

Results

NOTCH3-SVD stage Description N (%)
Stage {{ stageNames[stage] }} {{ stageDescriptions[stage] }} {{ frequency }} cases {{ (frequency / totalCaseCount * 100).toFixed(1) }}%


Global analysis of sequential versus non-sequential cases

The loaded cohort includes {{ typeDistribution['Sequential'] }} sequential cases ({{ (typeDistribution['Sequential'] / totalCaseCount * 100).toFixed(1) }}%, blue); {{ typeDistribution['Non-sequential'] }} non-sequential cases ({{ (typeDistribution['Non-sequential'] / totalCaseCount * 100).toFixed(1) }}%, red); and {{ typeDistribution['Deceased'] }} deceased cases ({{ (typeDistribution['Deceased'] / totalCaseCount * 100).toFixed(1) }}%, black).

In-depth analysis of sequential versus non-sequential cases

Legend:
⚫ = criterium for that stage is met
⚪ = criterium for that stage is not met
Combi-code = reference to the specific combination of met and unmet criteria which is also used in output file.
{{ caseType }} cases
Stage {{ stageNames[stage] }} N (%) NOTCH3-SVD stage Combi code CaseIDs
⚫ {{ (combination.wmh1) ? '⚫' : '⚪' }} ⚪ / ⚫ {{ (combination.wmh2) ? '⚫' : '⚪' }} ⚪ / ⚫ {{ (combination.lac1) ? '⚫' : '⚪' }} ⚪ / ⚫ {{ (combination.lac5) ? '⚫' : '⚪' }} ⚪ / ⚫ {{ (combination.mrs3) ? '⚫' : '⚪' }} ⚪ / ⚫ {{ (combination.mrs4) ? '⚫' : '⚪' }} ⚪ / ⚫ {{ (combination.mrs5) ? '⚫' : '⚪' }} ⚪ / ⚫ {{ (combination.deceased) ? '⚫' : '⚪' }} {{ combination.combinationCodeCount }} cases {{ combination.combinationCodePercentage }}% {{ stageNames[combination.N3SVD] }} {{ combination.combinationCode }} {{ patientData.filter(patient => (patient.combi === combination.combinationCode) || (combination.deceased && patient.combi > 128)).map(patient => patient.CaseID).join(', ') }}

Contact information

This online tool was developed as part of an international collaboration on disease staging in CADASIL and NOTCH3-associated small vessel disease. A paper describing this staging system was published in JAMA Neurology (https://doi.org/10.1001/jamaneurol.2024.4487).