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Isolation and Cannulation of Cerebral Parenchymal Arterioles
Published on: May 23, 2016
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Intracerebral hemorrhage in CADASIL
Shao-Lun Hsu1,2, Yi-Chu Liao3,4,5, Chih-Ping Chung3,4,5
1Institute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan, ROC.
Journal of the Chinese Medical Association : JCMA
|January 14, 2025
Summary
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) review shows intracerebral hemorrhage (ICH) is a key risk. Specific NOTCH3 mutations and hypertension increase ICH risk in CADASIL patients.
Area of Science:
- Neurology
- Genetics
- Vascular Medicine
Background:
- Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary small vessel disease.
- NOTCH3 gene mutations are the primary cause of CADASIL.
- Intracerebral hemorrhage (ICH) is an increasingly recognized, significant manifestation of CADASIL.
Purpose of the Study:
- To highlight intracerebral hemorrhage (ICH) as a major manifestation of CADASIL.
- To review risk factors, clinical features, and management of ICH in CADASIL.
- To emphasize the need for tailored management strategies for CADASIL patients at high risk for ICH.
Main Methods:
- Review of recent studies on CADASIL and intracerebral hemorrhage.
- Analysis of prevalence data, ethnic variations, and specific NOTCH3 mutations.
- Examination of neuroimaging findings, including cerebral microbleeds (CMBs).
Main Results:
- ICH prevalence in CADASIL ranges from 0.5% to 33.3%, influenced by ethnicity.
- Specific NOTCH3 mutations (e.g., p.R544C, p.R75P) and hypertension are linked to higher ICH risk.
- Cerebral microbleeds (CMBs) are strong predictors of ICH, predominantly occurring in the thalamus and basal ganglia.
Conclusions:
- CADASIL patients with ICH face higher morbidity, mortality, and stroke recurrence.
- Rigorous blood pressure control and cautious use of antithrombotics are crucial.
- Improved understanding of ICH in CADASIL can enhance patient outcomes.
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