The overall aim of this study is to investigate the pathophysiology of ICH by investigating intracerebral hemorrhage in HCHWA-D patients. By investigating triggers for ICH, hematoma expansion, recurrence rate and outcome in HCHWA-D related ICH we…
ID
Source
Brief title
Condition
- Neurological disorders congenital
- Central nervous system vascular disorders
- Vascular haemorrhagic disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
There are several primary study parameters. The main parameters are triggers of
ICH, presence of the *spot sign* on CT, hematoma progression on CT, amyloid and
microvascular changes on 3T/7T MRI, recurrence rate and clinical outcome.
Secondary outcome
Not applicable
Background summary
Although intracerebral hemorrhage (ICH) is a frequent subtype of stroke, the
research in this field has received far less attention than ischemic stroke.
One frequent cause of lobar hemorrhage in the elderly is sporadic cerebral
amyloid angiopathy (sCAA). sCAA is characterized by the deposition of amyloid-*
peptide and degenerative changes in the capillaries, arterioles, and small and
medium sized arteries of the cerebral cortex, leptomeninges, and cerebellum.
Hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D) is an
autosomal dominant form of CAA, in which the amyloid angiopathy is
pathologically and biochemically similar to sCAA. The disease is characterized
by (repeated) intracerebral hemorrhage and cognitive decline. Since in patients
with HCHWA-D the genetic background is known it offers a unique opportunity to
investigate in vivo the role of these vascular amyloid depositions on ICH
progression, recurrence rate and outcome.
Study objective
The overall aim of this study is to investigate the pathophysiology of ICH by
investigating intracerebral hemorrhage in HCHWA-D patients. By investigating
triggers for ICH, hematoma expansion, recurrence rate and outcome in HCHWA-D
related ICH we expect not only to better understand HCHWA-D related ICH but
also to increase insight in the pathophysiology of sCAA related ICH.
Study design
Our study is an observational cohort study. The design of the studies
considering triggers, the spot sign, hematoma expansion, and 3T/7T MRI are
prospective, the design of the study considering recurrence rate and final
outcome is retrospective. The study on triggers of ICH has a case-cross over
design.
Study burden and risks
The potential risks are limited. The risk of the additional CT scan is a low
dosage of radiation (the radiation dose of one non-contrast CT is 0,8 to 1,1
mSv). The risks of MRI are minimal (risk of every day life), because there are
no known consequences to the health of the participant. The mutation carriers
may benefit from more insight into hemorrhages related to their disease,
especially the part on triggers and hematoma growth. Patients with sCAA are
needed as comparison for patients with HCHWA-D. By investigating possible
modifying factors such as hypertension, coagulation factors and cholesterol we
hopefully will discover new targets for treatment of ICH in patients with
HCHWA-D. Moreover, this study could eventually lead to much more insights about
both sCAA and pICH in general and will be of importance to define endpoints for
future intervention trials.
Albinusdreef 2
Leiden 2300 RC
NL
Albinusdreef 2
Leiden 2300 RC
NL
Listed location countries
Age
Inclusion criteria
HCHWA-D patients
1. Age * 18y
2. Written informed consent from the patient or in case the patients is not able to give informed consent from his/her legal representative (see appendix 2 for the evaluation of the (mental) ability of the patient to give informed consent).
3. Presence of the amyloid precursor protein codon 693 mutation or a hemorrhage pattern on CT or MRI suspect for amyloid angiopathy and first degree relative with HCHWA-D.
sCAA patients
1. Age * 18y
2. Written informed consent from the patient or in case the patients is not able to give informed consent from his/her legal representative (see appendix 2 for the evaluation of the (mental) ability of the patient to give informed consent).
3. Probable CAA based on the modified Boston criteria and no family history of HCHWA-D (see appendix 1) 15 In case there is doubt about a possible familiar type of sCAA a DNA test will be offered to the patients to test for HCHWA-D.
Exclusion criteria
* Age < 18 years
* For the 3T and 7T MRI study:
- Claustrophobia
- Renal failure (only for the contrast enhanced substudy of 7T MRI)
Possible other contra-indications for MRI (for example pacemakers and defibrillators, nerve stimulators, intracranial clips, intraorbital or intraocular metallic fragments, cochlear implants, ferromagnetic implants, hydrocephalus pump, intra-uterine device, an iron wire behind the teeth, permanent make-up, tattoos above the shoulders) will be discussed by the investigator with the MRI technician under supervision of professor A. Webb of the C.J. Gorter Center for High-field MRI.
Design
Recruitment
metc-ldd@lumc.nl
metc-ldd@lumc.nl
metc-ldd@lumc.nl
metc-ldd@lumc.nl
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 | NL44719.058.13 |