To determine whether pathological changes are visible in brain parenchyma, blood vessels or other intracranial structures on ultra high field MRI in SCD patients in normal anatomical areas on 3.0 Tesla MRI and to determine the nature of these…
ID
Source
Brief title
Condition
- Haemoglobinopathies
- Blood and lymphatic system disorders congenital
- Central nervous system vascular disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
The main endpoint is the presence of (pre-) pathological changes of brain
parenchyma, blood vessels or other intracranial structures at 7.0 Tesla MRI.
Intracerebral changes include cystic infarction, atrophy, encephalomalacia,
leukoencephalopathy or changes of the intracerebral blood vessels.
Secondary outcome
Demographical and clinical patient characteristics including age, HbS
phenotype, Hb, HbF, leukocytes during stable clinical state, presence of
epilepsy.
Cerebral blood flow asymmetry, defined as previously described by Van den Tweel
et al.
Neurological examination.
Verbal IQ, performance IQ and full scale IQ, and visuo-motor functioning.
Background summary
Sickle cell disease is a hereditary hemoglobinopathy which causes chronic
hemolysis and vaso-occlusion leading to irreversible damage in multiple organs.
Silent braininfarcts (braininfarction without overt neurological deficits) can
be seen on conventional MRI in 22-25% of children. These silent infarcts are
associated with an increased risk for further infarcts, neuropsychological
dysfunction and impairment of cognitive development.
Little is known about the exact etiology and risk factors of silent infarcts,
however, early recognition of patients at high risk of silent infarction is of
great importance for adequate prevention and treatment. A previous study by the
AMC research group on SCD indicated that children with SCD without overt
infarctions have left to right asymmetries in cerebral perfusion, this could
represent an early stage of pathology in which intervention might prevent
further neurologic damage.
Therefore, the purpose of this study is to evaluate the extent and nature of
brain parenchymal pathology in patients with SCD without overt infarcts using
high field MRI and to investigate a possible association with cerebral blood
flow asymmetry and neurocognitive functioning.
Study objective
To determine whether pathological changes are visible in brain parenchyma,
blood vessels or other intracranial structures on ultra high field MRI in SCD
patients in normal anatomical areas on 3.0 Tesla MRI and to determine the
nature of these changes.
To evaluate the association between cerebral blood flow asymmetry and
pathological changes of brain parenchyma on ultra high field MRI.
To evaluate the association between neurological examination, neurocognitive
functioning and pathological changes of brain parenchyma on ultra high field
MRI.
To compare rates of pathological changes visible on 3.0 Tesla and 7.0 Tesla MRI
scanning..
Study design
Observational cohort study.
Study burden and risks
The risk of participation in this study is negligible; there are no known
side-effects of performing a MRI scan. IIt is usual for patients with sickle
cell disease to undergo blood sampling, ultrasonography or MR imaging of the
brain. The risks and burden associated with participation in this study are
thus comparable to risks and burden of everyday life in patients with sickle
cell disease.
Meibergdreef 9
1105 AZ Amsterdam
NL
Meibergdreef 9
1105 AZ Amsterdam
NL
Listed location countries
Age
Inclusion criteria
patients with sickle cell disease
treated at AMC
aged 18-25
Exclusion criteria
Overt stroke, defined as a focal neurological deficit with either motor or sensory deficit lasting more than 24 hr or focal neurological deficit lasting less than 24 hr with neuroimaging evidence of a cerebral infarct corresponding with the focal deficit.
Chronic blood transfusion schedule.
The presence of metal in the body (e.g. osteosynthetic material, pacemaker, artificial cardiac valves).
Claustrophobia.
Surgery performed in the area of measurement in the last 3 months.
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 | NL33730.018.10 |