Current Understanding of Pathology and Therapeutic Status for CADASIL

  • Suning Ping
  • Li-Ru ZhaoEmail author
Part of the Springer Series in Translational Stroke Research book series (SSTSR)


Recently, it has been drawn an increased attention on a hereditary form of stroke and vascular dementia named cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Because of poor understanding of the pathogenesis of this disease, the treatment that specifically delay or stop the pathological progression of CADASIL has not yet been developed. This chapter provides an update on CADASIL research. The pathological features and possible molecular mechanisms of CADASIL are outlined, the involvement of vascular endothelial cells in pathological progression of CADASIL is introduced, and emerging studies in treatment research for CADASIL is reviewed and discussed.


CADASIL Notch3 mutation Vascular smooth muscle cells Endothelial cells Capillary thrombosis Pathology Treatment 



Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy


Epidermal growth factor-like repeats


Granulocyte colony stimulating factor


Granular osmiophilic materials


Migraine with aura


NOTCH3 N-terminal extracellular domain


NOTCH3 transmembrane intracellular domain


Stem cell factor


Vascular smooth muscle cells



This work was supported by American Heart Association (15GRNT25700284).

Funding information This work was supported by American Heart Association (15GRNT25700284).


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© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Neurosurgery, State University of New YorkUpstate Medical UniversitySyracuseUSA

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