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@ARTICLE{Stringer:276782,
      author       = {Stringer, Michael S and Blair, Gordon W and Kopczak, Anna
                      and Kerkhofs, Danielle and Thrippleton, Michael J and
                      Chappell, Francesca M and Maniega, Susana Muñoz and Brown,
                      Rosalind and Shuler, Kirsten and Hamilton, Iona and Garcia,
                      Daniela Jaime and Doubal, Fergus N and Clancy, Una and
                      Sakka, Eleni and Poliakova, Tetiana and Janssen, Esther and
                      Duering, Marco and Ingrisch, Michael and Staals, Julie and
                      Backes, Walter H and van Oostenbrugge, Robert and Biessels,
                      Geert Jan and Dichgans, Martin and Wardlaw, Joanna M},
      collaboration = {consortium, SVDs@target},
      title        = {{C}erebrovascular {F}unction in {S}poradic and {G}enetic
                      {C}erebral {S}mall {V}essel {D}isease.},
      journal      = {Annals of neurology},
      volume       = {97},
      number       = {3},
      issn         = {0364-5134},
      address      = {Hoboken, NJ},
      publisher    = {Wiley-Blackwell},
      reportid     = {DZNE-2025-00319},
      pages        = {483 - 498},
      year         = {2025},
      abstract     = {Cerebral small vessel diseases (SVDs) are associated with
                      cerebrovascular dysfunction, such as increased blood-brain
                      barrier leakage (permeability surface area product),
                      vascular pulsatility, and decreased cerebrovascular
                      reactivity (CVR). No studies assessed all 3 functions
                      concurrently. We assessed 3 key vascular functions in
                      sporadic and genetic SVD to determine associations with SVD
                      severity, subtype, and interrelations.In this prospective,
                      cross-sectional, multicenter INVESTIGATE-SVDs study, we
                      acquired brain magnetic resonance imaging in patients with
                      sporadic SVD/cerebral autosomal dominant arteriopathy with
                      subcortical infarcts and leukoencephalopathy (CADASIL),
                      including structural, quantitative microstructural,
                      permeability surface area product, blood plasma volume
                      fraction, vascular pulsatility, and CVR (in response to CO2)
                      scans. We determined vascular function and white matter
                      hyperintensity (WMH) associations, using covariate-adjusted
                      linear regression; normal-appearing white matter and WMH
                      differences, interrelationships between vascular functions,
                      using linear mixed models; and major sources of variance
                      using principal component analyses.We recruited 77 patients
                      (45 sporadic/32 CADASIL) at 3 sites. In adjusted analyses,
                      patients with worse WMH had lower CVR (B = -1.78, $95\%$ CI
                      -3.30, -0.27) and blood plasma volume fraction (B = -0.594,
                      $95\%$ CI -0.987, -0.202). CVR was worse in WMH than
                      normal-appearing white matter (eg, CVR: B = -0.048, $95\%$
                      CI -0.079, -0.017). Adjusting for WMH severity, SVD subtype
                      had minimal influence on vascular function (eg, CVR in
                      CADASIL vs sporadic: B = 0.0169, $95\%$ CI -0.0247, 0.0584).
                      Different vascular function mechanisms were not generally
                      interrelated (eg, permeability surface area product~CVR: B =
                      -0.85, $95\%$ CI -4.72, 3.02). Principal component analyses
                      identified WMH volume/quantitative microstructural metrics
                      explained most variance in CADASIL and arterial pulsatility
                      in sporadic SVD, but similar main variance sources.Vascular
                      function was worse with higher WMH, and in WMH than
                      normal-appearing white matter. Sporadic SVD-CADASIL
                      differences largely reflect disease severity. Limited
                      vascular function interrelations may suggest disease
                      stage-specific differences. ANN NEUROL 2025;97:483-498.},
      keywords     = {Humans / Female / Male / Middle Aged / Cerebral Small
                      Vessel Diseases: genetics / Cerebral Small Vessel Diseases:
                      diagnostic imaging / Cerebral Small Vessel Diseases:
                      physiopathology / Cross-Sectional Studies / Aged / Magnetic
                      Resonance Imaging / Prospective Studies / CADASIL: genetics
                      / CADASIL: physiopathology / CADASIL: diagnostic imaging /
                      Cerebrovascular Circulation: physiology / White Matter:
                      diagnostic imaging / White Matter: pathology / Adult},
      cin          = {AG Dichgans},
      ddc          = {610},
      cid          = {I:(DE-2719)5000022},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:39552538},
      pmc          = {pmc:PMC11831873},
      doi          = {10.1002/ana.27136},
      url          = {https://pub.dzne.de/record/276782},
}