Reversible Cerebral Vasoconstriction Syndrome Clinical Trial
Official title:
Perfusion MRI in Reversible Cerebral Vasoconstriction Syndrome
This study aims to quantify perfusion, assess arterial vasoconstriction, and confirm reversibility using 3T ASL-MRI and MRA in 10 patients with suspected RCVS. Acquiring these data at multiple time points during RCVS progression, the investigators will assess the relationship between vasoconstriction and downstream perfusion and determine the role of these imaging techniques in early and accurate diagnosis of RCVS. The investigators also aim to investigate whether early imaging abnormalities can predict RCVS complications and clinical outcomes.
Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a group of conditions characterised
by prolonged but reversible multifocal narrowing of the cerebral arteries. It presents
typically as acute severe headache, usually recurrent and thunderclap in character, with or
without additional symptoms and signs. Adverse complications associated with RCVS can be
devastating especially if not recognised early; depending on the degree of vasoconstriction,
RCVS may be associated with cortical subarachnoid haemorrhage (in approximately 34% of
patients), ischaemic infarction (6-39% of patients), or concomitant posterior reversible
encephalopathy syndrome (PRES, 9-38% of patients). RCVS may also present as parenchymal
brain haemorrhage in 20% of cases. The data on complications rate highlight the uncertainty
about the condition and indicate need for more research to better characterise the evolution
of the pathology; hence need for this study as it is prospective and longitudinal.
The hallmark of RCVS is vasoconstriction seen on vascular imaging scans and typically
reverses within 3 months. Prevalence of radiological vasoconstriction seen on magnetic
resonance angiography (MRA) in RCVS is reported to be between 60-90% and typically appears
as diffuse segmental constriction of large and medium sized vessels lasting 4-12 weeks. The
main advantage of MRA is that it can be performed without the use of a radioactive tracer,
thus providing a safe method for repeat observations of vascular pathology. Imaging is often
negative in first 4-5 days following the onset of headache; The mean time to detect
abnormality on vascular imaging has been reported as 8 days after headache onset. RCVS
symptoms usually resolves by 1 month after presentation, however the adverse complications
associated with RCVS may have lasting consequences as described above. Magnetic resonance
imaging (MRI) is an excellent tool for characterising brain changes during the progression
and resolution of RCVS. Standard structural images can identify complications of RCVS, such
as bleeding, ischaemia, and PRES.
Finally, Arterial Spin Labeling (ASL) MRI can be used to non-invasively quantify perfusion
of brain tissue, providing a measure of the impact of upstream arterial vasoconstriction on
local cortical regions.
Cortical perfusion has not yet been extensively studied in RCVS; at time of writing, only
two case reports have been published. Rosenbloom and Singhal reported a case of RCVS induced
by carotid endarterectomy following a frontal lobe ischaemic stroke. Perfusion MRI showed
unilateral hypo-perfusion, mainly affecting internal watershed areas with superficial
cortical regions being relatively spared. In a second study, ASL-MRI was performed on a
50-year-old man with RCVS who presented with severe recurrent headaches and neurological
deficits (localising to the right hemisphere). ASL-MRI demonstrated significant
hypo-perfusion in the right parieto-occipital lobe, but no infarct was seen on diffusion
imaging. At 12 weeks, there was complete resolution of cerebral vasoconstriction on
angiography and normal perfusion findings on ASL-MRI.
These case studies suggest that perfusion MRI can offer an additional tool to confirm and
understand RCVS. ASL-MRI is a non-invasive, radiation and contrast-free technique that can
be performed at multiple time points to monitor changes in perfusion over the time period of
RCVS resolution and assess response to potential therapeutics.
One of the disadvantages of ASL-MRI is a low signal to noise ratio, this can be addressed by
using high-field MRI at 3 Tesla (3T). In addition, 3T MRI can provide very good spatial
resolution. The University of Nottingham represents one of the leading international
research centres with experience in using high and ultra-high field MRI for investigating
different neurological diseases such as multiple sclerosis and brain tumours with excellent
results. Applying advanced non-invasive MRI techniques in this study will be a significant
advantage as we investigate RCVS, understand the pathophysiology, and assess brain perfusion
in multiple time points.
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Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Diagnostic
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