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@ARTICLE{Zhang:285357,
      author       = {Zhang, Ruiting and Chen, Chih-Hao and Lambert, Louis and
                      Cheng, Yu-Wen and Lebenberg, Jessica and Tezenas Du Montcel,
                      Sophie and Hervé, Dominique and Guey, Stephanie and
                      Dichgans, Martin and Tang, Sung-Chun and Chabriat, Hugues},
      title        = {{S}imple {MRI} {L}esion {L}evels {I}mprove {T}wo-{Y}ear
                      {P}rognostic {A}ccuracy {B}eyond {C}linical {H}istory in
                      {CADASIL}.},
      journal      = {Stroke},
      volume       = {57},
      number       = {3},
      issn         = {0039-2499},
      address      = {New York, NY},
      publisher    = {Association},
      reportid     = {DZNE-2026-00223},
      pages        = {758 - 769},
      year         = {2026},
      abstract     = {Cerebral autosomal dominant arteriopathy with subcortical
                      infarcts and leukoencephalopathy (CADASIL) is the most
                      common hereditary cerebral small-vessel disease. The CADASIL
                      MRI Inventory Tool summarizes individual MRI findings as
                      simple, type-specific lesion levels. We assessed the
                      predictive value of these levels beyond clinical
                      information.At baseline, CADASIL MRI Inventory Tool levels
                      were assigned for periventricular, deep, and superficial
                      white matter hyperintensities, lacunes, cerebral
                      microbleeds, perivascular spaces in the centrum semiovale
                      and basal ganglia, superficial and deep atrophy, large
                      infarcts, and macrobleeds. Outcomes included stroke,
                      migraine with aura (MA), moderate or severe cognitive
                      impairment, and disability, which were assessed at baseline
                      and during 2-year follow-up. Multivariable logistic
                      regression was performed, with adjustment for age, sex,
                      vascular risk factors, mutation location, and education
                      level.We analyzed 743 patients from France, Germany, and
                      Taiwan (mean age, 53±12; $55\%$ with prior stroke; $35\%$
                      with MA; $15\%$ with disability; $18\%$ with cognitive
                      impairment). At baseline, higher deep white matter
                      hyperintensities and lacune levels were associated with
                      stroke, whereas cerebral microbleeds and superficial atrophy
                      were inversely associated with MA. Superficial atrophy and
                      periventricular white matter hyperintensities were higher,
                      and superficial white matter hyperintensities were lower,
                      among those with cognitive impairment, whereas deep atrophy
                      and lacunes were linked to disability. Over 2 years (n=547),
                      $11.7\%$ experienced ischemic stroke events, $22.2\%$ MA
                      attacks, $6.5\%$ developed disability, and $6.9\%$ moderate
                      or severe cognitive impairment. After adjustment, lacune
                      levels independently predicted ischemic stroke events (odds
                      ratio, 6.10 $[95\%$ CI, 2.09-26.02] for all levels
                      combined). Superficial atrophy and higher superficial white
                      matter hyperintensities predicted a lower risk of MA (odds
                      ratio, 0.29 and 0.21 $[95\%$ CI, 0.12-0.68] and
                      [0.06-0.77]). Lacunes and deep atrophy predicted disability,
                      and lacunes with superficial atrophy predicted cognitive
                      impairment.CADASIL MRI Inventory Tool lesion levels are
                      differentially associated with CADASIL manifestations and
                      provide independent 2-year prognostic information beyond
                      clinical covariates. These simple measures may supplement
                      clinical evaluation to improve short-term risk
                      stratification and support patient selection in clinical
                      trials.},
      keywords     = {Humans / CADASIL: diagnostic imaging / CADASIL: pathology /
                      Male / Female / Middle Aged / Magnetic Resonance Imaging:
                      methods / Prognosis / Adult / Aged / White Matter:
                      diagnostic imaging / White Matter: pathology / Stroke /
                      Cognitive Dysfunction / Atrophy / NOTCH3 mutation (Other) /
                      cerebral atrophy (Other) / ischemic stroke (Other) / lacune
                      (Other) / longitudinal (Other) / microbleed (Other) / white
                      matter hyperintensities (Other)},
      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:41439314},
      doi          = {10.1161/STROKEAHA.125.053727},
      url          = {https://pub.dzne.de/record/285357},
}