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Prolonged cerebral transit time in CADASIL: a transcranial ultrasound study
Martin Liebetrau1, Jürgen Herzog, Christian U A Kloss
1Department of Neurology, Klinikum Grosshadern, Ludwig-Maximilians University, München, Germany.
Insights
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) shows prolonged cerebral transit time (CTT). This finding may help detect CADASIL microvascular changes in clinical settings.
Area of Science:
- Neurology
- Vascular Biology
- Medical Imaging
Background:
- Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetic small vessel disease.
- CADASIL is characterized by microvascular accumulation of granular osmophilic material.
- Notch3 gene mutations are the cause of CADASIL.
Purpose of the Study:
- To measure arteriovenous cerebral transit time (CTT) in CADASIL patients.
- To identify microangiopathy-related changes in CADASIL using CTT.
- To assess the clinical utility of CTT in detecting small vessel disease.
Main Methods:
- Transcranial color-coded duplex sonography was used to measure CTT.
- Ultrasound contrast agent Levovist was administered to 17 CADASIL patients and 17 controls.
- CTT was recorded from the posterior cerebral artery to the vein of Galen.
Main Results:
- Mean CTT was significantly prolonged in CADASIL patients (4.4±1.9s) compared to controls (1.3±0.5s).
- This prolongation was evident even in non-disabled CADASIL individuals.
- A trend for correlation between CTT and clinical disability (Rankin score) was observed.
Conclusions:
- Prolonged CTT indicates microvascular alterations in CADASIL.
- CTT measurement is a potential clinical tool for diagnosing small vessel diseases.
- Further studies are needed to differentiate CTT in various small vessel diseases.
Background And Purpose:
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary angiopathy caused by mutations in Notch3. Cerebral microvessels show an accumulation of granular osmophilic material in the vicinity of degenerating vascular smooth muscle cells. In this study, we measured the arteriovenous cerebral transit time (CTT) to identify changes related to the microangiopathy in CADASIL.
Methods:
CTT is the time that a contrast agent needs to pass from a cerebral artery to its corresponding vein. CTT was measured in 17 CADASIL individuals (mean age, 50.2+/-12.3 years) and an equal number of age- and sex-matched control subjects (mean age, 48.9+/-13.0 years) with transcranial color-coded duplex sonography. The intensity curves were recorded in the P2 segment of the posterior cerebral artery and the vein of Galen after injection of the ultrasound contrast agent Levovist.
Results:
CTT was significantly prolonged in individuals with CADASIL (4.4+/-1.9 seconds) compared with control subjects (1.3+/-0.5 seconds, P<0.0001). This difference was also significant when only nondisabled CADASIL individuals (Rankin score=0, n=9) were analyzed (P<0.0001). There was a nonsignificant trend for a correlation between Rankin score and CTT (r=0.39, P=0.11).
Conclusions:
The prolonged CTT likely reflects microvascular changes in CADASIL. Measurements of the CTT may be used clinically to disclose small-vessel disease. Studies comparing CADASIL subjects with other patient populations seem warranted to determine possible differences in CTT between different types of small-vessel disease.