The aim of this study is to invite a new cohort of CADASIL patients for a 7 Tesla MRI scan. By doing this, we aim to get more insight into the pattern and development of cortical atrophy and vessel wall thickening in CADASIL patients of different…
ID
Source
Brief title
Condition
- Congenital and hereditary disorders NEC
- Central nervous system vascular disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
- Average thickness of the specific cortical layers, compared to those of the
control subjects
- Focal abnormalities in the specific cortical layers
- Average wall thickness of the medial meningeal artery and the relation with
patient age, compared to control subjects
- Shape of SLLs and relation with blood vessels
- Number of microbleeds and age distribution
Secondary outcome
n/a
Background summary
CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and
leukoencephalopathy) is a dominantly inherited disease of the vessel walls
which mainly manifests in the central nervous system and which leads to
recurrent cerebral infarcts, migraine with aura and cognitive decline at young
adult age to middle age. Imaging of the brain shows white matter
hyperintensities, subcortical infarcts, microbleeds, subcortical lacunar
lesions (SLLs) and cortical atrophy. Postmortal pathologic studies have shown
that the cortical atrofy is mainly present in cortical layers 3 and 5, and that
vessel wall thickening is present mainly in arterioles and leptomeningeal
vessels. It has not been possible yet to demonstrate these findings in vivo.
A 7 Tesla MRI-scanner has become available in the LUMC in 2007. The most
important advantages of this scanner, compared to the current 1.5 Tesla and 3.0
Tesla scanners, are: an increased spatial resolution and an increased
sensitivity for susceptibility artifacts, resulting in an improved detection
rate for diagnosing very small microbleeds.
Study objective
The aim of this study is to invite a new cohort of CADASIL patients for a 7
Tesla MRI scan. By doing this, we aim to get more insight into the pattern and
development of cortical atrophy and vessel wall thickening in CADASIL patients
of different ages. We also aim to make a better assessment of the prevalence,
location and quantity of microbleeds in CADASIL and we aim to determine the
exact anatomical substrate of the SLLs.
Study design
Patients will undergo an MRI scan of the brain on the 7 Tesla MRI scanner. By
using a special surface coil, the level of cortical atrophy and leptomeningeal
vessel wall thickening will be studied. Additionally, the subcortically located
SLLs will be studied, and a characterisation of the microbleeds will take
place.
Study burden and risks
This is an obvervational study, without invasive procedures. The study does not
have any medical risks. Burden of participation for the participants will
mainly consist of travelling to Leiden and cooperating with the MRI scan.
Albinusdreef 2
2333ZA Leiden
Nederland
Albinusdreef 2
2333ZA Leiden
Nederland
Listed location countries
Age
Inclusion criteria
Confirmed NOTCH3 mutation
Age: 18 years or older
Mental competency
Exclusion criteria
MRI contraindications
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL25553.058.08 |