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Spontaneous intracerebral hemorrhage in CADASIL
Lifei Lian, Dujuan Li, Zheng Xue
1Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1095 Jiefang Ave, Wuhan, Hubei 430030, P,R, China. zhusuiqiang@gmail.com.
The Journal of Headache and Pain
|December 19, 2013
Summary
Intracerebral hemorrhage (ICH) is rare in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL). This case highlights ICH risk in CADASIL patients, even without hypertension or antithrombotics.
Area of Science:
- Neurology
- Genetics
- Vascular Medicine
Background:
- Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a rare genetic small vessel disease.
- Ischemic events are the primary clinical manifestation, while spontaneous intracerebral hemorrhage (ICH) is exceptionally uncommon.
Observation:
- A 46-year-old Chinese man with diagnosed CADASIL developed a large basal ganglia hematoma.
- The patient was normotensive, not on antithrombotics, and had pre-ICH multiple cerebral microbleeds (CMBs) on MRI.
- Migraine occurred 10 months post-ICH.
Findings:
- This is the first reported case of ICH in a CADASIL patient with the Arg90Cys mutation in exon 3.
- The presence of CMBs may indicate an increased risk for ICH in CADASIL.
Implications:
- ICH should be considered in CADASIL patients presenting with new neurological events.
- MRI screening for CMBs could aid in predicting ICH risk and guiding antithrombotic therapy.
- Strict blood pressure control and judicious use of antithrombotics are crucial for managing CADASIL patients.
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