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eEdE-41 - The Many Presentations of
Reversible Cerebral Vasoconstriction
Syndrome (RCVS), A Great Masquerader
Katyucia De Macedo Rodrigues, Eduardo Scortegagna Jr.,
Deepak Takhtani, Ajit Puri, Sathish Dundamadappa, Rania Hito
University of Massachusetts Medical School
Disclosure
The authors have no relevant
conflict of interest to declare
Purpose
Classically presenting as a thunderclap headache,
RCVS may mimic a number of different entities,
making the initial diagnosis challenging in the
absence of high clinical suspicion.
Our aim is to present a case based review of
RCVS, demonstrating common and uncommon
imaging findings on CT, MRI, CT angiography
(CTA), MR angiography (MRA) and conventional
digital subtraction angiography (DSA).
What is RCVS?
The precise pathophysiology of Reversible Cerebral
Vasoconstriction Syndrome (RCVS) remains unclear. It is
believed that a transient disorder of vascular tone
regulation leads to inappropriate arterial constrictions,
which occur in a segmental fashion affecting multiple
arteries in different vascular territories. An inciting factor
may or may not be identified. Sympathomimetic or
vasoactive
agents,
including
amphetamines,
phenylpropanolamine, pseudoephedrine, serotonergic
antidepressants, nicotine, caffeine, cannabis, and triptan
or ergot-containing medications are among common
triggers.
Click here for a comprehensive list of inciting factor associated with RCVS
Historical Background
• RCVS is a unifying term for a manifestation that may
occur in different clinical settings. It encompasses the
previously described “Call-Fleming syndrome”,
“postpartum angiopathy”, “migrainous vasospasm”,
“migraine angiitis”, “drug-induced angiopathy”, “benign
angiopathy of the central nervous system (CNS)”, and
“CNS pseudovasculitis”.
• In 2007, Calabrese et al proposed the term RCVS to
unify the common vascular manifestation of these
different entities, coursing with similar clinical,
laboratory, and angiographic findings. Specific
diagnostic criteria were also suggested.
Diagnostic Criteria
• Multiple segmental vasoconstrictions on vascular
imaging studies;
• Aneurysmal hemorrhage is excluded;
• Normal or near normal CSF (protein level 􏰀 80
mg%, leukocytes 􏰀10 mm3, normal glucose level);
• Severe headache with or without other
neurological manifestation;
• Resolution of vasoconstrictions within 12 weeks.
Clinical presentation
• Patients most commonly present with severe headache that
escalates in a few seconds (thunderclap headaches). It usually
persists for days before a diagnosis is made.
• Patients with prior history of migraines usually report a headache
that is different in quality, intensity or location as compared to prior
migraine episodes.
• Symptoms related to complications, such as stroke or TIA, can be
present, usually manifesting within 1 to 2 weeks.
• There is a common association between PRES and RCVS, likely
related to the vasoactive tone deregulation on both entities, with
unclear boundaries between the two processes. Approximately 85%
of patients with PRES demonstrate some degree of vasoconstriction
on vascular imaging.
CASE BASED REVIEW OF
RADIOLOGICAL FINDINGS
Case 1
26 year old female sprayed with pepper spray on face and eyes,
developed severe headache several hours later
A
B
A - NCCT, B - FLAIR and C - Diffusion weighted imaging (DWI): Initial non vascular imaging
shows no significant abnormality.
C
A
C
B
A: CTA image showing multiple segmental
vasoconstrictions along the right middle
cerebral artery (MCA) branches. B: Magnified
view of A with arrows pointing at arterial
constrictions. C: MRA shows multiple small
segmental vasoconstrictions along both
posterior cerebral arteries.
A
B
Workup for vasculitis
negative. Patient
showed dramatic
improvement of
headaches after oral
administration of
verapamil. Discharged
asymptomatic with no
recurrence of symptom
at 4 and 44 month
follow up.
A: DSA shows multiples segmental
vasoconstriction in branches of right
MCA, right posterior cerebral artery
(PCA) and anterior cerebral artery (ACA).
Black arrows show some of the
vasoconstrictions. White box show area
magnified in B. B: Black arrows show
vasoconstriction in ACA branches. C: DSA
showing multiple segmental narrowing
along both PCAs. D: Magnified view of C,
showing vasoconstrictions of distal left
PCA (arrows).
C
D
Radiological findings
• Conventional imaging in the absence of
complication is typically normal. Initial vascular
imaging may be normal in the first week. A high
clinical suspicion is imperative for pursuing
additional radiological evidence of transient
vasoconstriction.
• In typical clinical presentations with normal initial
imaging, a provisional diagnosis of possible RCVS
should be considered until follow up images
confirm or exclude the diagnosis.
Case 2
50 year old male with prior history of migraine headaches
and post coital headaches, presenting with severe
headache worsening in the last 2 days
A
B
C
D
A: NCCT shows subtle sulcal subarachnoid hemorrhage (SAH) in the left cerebral convexity
(arrow), which is demonstrated in the sagittal reformat in B. C: Axial FLAIR showing high
signal within the left central sulcus, confirming the presence of SAH, which is also
demonstrated on the susceptibility weighted angiography images (SWAN) .
A
B
A: Initial MRA shows multiple segmental arterial narrowing (arrows) involving
branches of bilateral MCAs and PCAs. B: 2 month followup MRA showing
resolution of vasoconstrictions with oral treatment with of calcium channel
blocker.
Radiological findings
• RCVS can present with subarachnoid hemorrhage
(SAH) that is usual sulcal in the cerebral convexities.
• SAH related to ruptured aneurysm is more often
centered in basal cisterns and around the circle of
Willis.
• Non aneurysmal idiopathic hemorrhage is centered in
the perimesencephalic cistern.
• Cortical Venous thrombosis can present with sulcal SAH
in the cerebral convexities, similar to the appearance of
RCVS. Additional clinical information and radiological
studies may be necessary to establish a diagnosis.
Radiological Findings
Role of imaging studies:
• Demonstrate
vasoconstriction;
multifocal
segmental
• Exclude possible alternative diagnosis, such as
primary angiitis of the central nervous system
(PACNS), aneurysmal bleed, venous thrombosis
and amyloid angiopathy;
• Evaluate for possible complications, such as
infarct, intracranial bleed and parenchymal
edema.
Case 3
56 year old male with right leg
weakness and right arm paresthesia
A
B
C
D
Axial DWI showing small cortical infarct in the medial aspect of the posterior right frontal lobe in A and
in the deep white matter of the left frontal lobe in B. CTA shows narrowing of the M1 segment of the
left MCA in C and multifocal segmental narrowing of the left MCA branches in D.
A
B
DSA before (A) and after (B) intra-arterial (IA) administration of
verapamil showing improvement of arterial narrowing.
Patient maintained on oral verapamil and discharged home with
resolution of symptoms.
Radiological findings
• RCVS may evolve with symptoms related to
stroke or TIA.
• Should be suspected in patients with typical
history and stroke involving multiple vascular
territories in the absence of significant
atheromatous disease or identifiable embolic
source.
• Infarcts usually in watershed zones.
Case 4
48 year old female with worst headache of her life,
vomiting and photophobia several hours after being hit
on face mask by a paintball
A
B
C
A: Axial FLAIR demonstrates increased signal within the sulci of the high cerebral convexities. B:
Axial DWI fails to show restricted diffusivity correlating with FLAIR abnormal signal. C: Axial post
intravenous contrast T1 weighted-images demonstrate increased enhancement correlating with
FLAIR signal abnormality.
A
B
CTA demonstrate segmental vasoconstrictions in ACAs branches (A)
and right MCA branches (B). Note distal caliber greater than
proximal, instead of expected normal smooth tapering of vessel
caliber.
A
B
DSA of right internal carotid artery (RICA) before (A) and after (B) the IA
administration of verapamil. Note the significant interval improvement of
arterial vasoconstrictions.
Lumbar puncture was negative for SAH. Patient discharged home on oral verapamil
without symptoms or deficits.
Radiological findings
• Hyperintense vessel sign on FLAIR is one of
the possible presentations of RCVS.
• It mimics high FLAIR signal seen in cases of
SAH, inflammatory meningeal processes or
leptomeningeal spread of tumor.
• High signal is related to slow flow within
affected branches.
• CSF analysis typically negative for SAH.
Case 5
59-year-old woman admitted for 5 days due to Crohn's
disease flare, developing sudden onset of loss of vision in
both eyes.
A
B
C
A: Axial FLAIR images demonstrates increased signal within the occipital lobes,
corresponding cytotoxic edema on the DWI (B) and ADC map (C). Findings
raised suspicion for posterior reversible encephalopathy syndrome (PRES).
A
B
CTA angiogram shows multifocal vasoconstrictions in the
right MCA and bilateral ACA branches. The possibility of
vasculitis was also raised.
A
B
DSA of RICA before (A) and after (B) the IA administration of
verapamil
shows
significant
interval
improvement
of
vasoconstrictions.
Patient discharged home with symptoms related to occipital strokes –
severe bilateral visual loss - with a diagnosis of RCVS.
Follow up MR angiogram
40 days later shows no
residual vasospasm.
Radiological findings
• There is significant overlap between PRES and
RCVS with PRES-like reversible cerebral edema
encountered in 9% to 38% of patients with
RCVS, while most patients with PRES (􏰀85%)
demonstrate some element of RCVS-like
cerebral vasoconstriction when conventional
angiography is performed.
• Distinction between the two entities may not
be possible due to similar causative disorders.
Radiological findings
• Primary Angiitis of the Central Nervous System (PACNS)
affects older men and demonstrate abnormal CSF analysis,
with increased white blood cell count and protein.
• Thunderclap headache is rarely associated with PACNS.
Instead, patients complain of insidious pain, which is
chronic in duration.
• On imaging, PACNS will often demonstrate multiple infarcts
of different ages and has a low rate of hemorrhagic
complications.
• Association with PRES is also unusual for PACNS.
• Differentiation between PACNS and RCVS is imperative,
giving significant differences in management and prognosis.
Case 6
59 year old female presenting with severe
headaches and vomiting. Drug screen positive
for citalopram and pseudoephedrine
A
B
C
A: NCCT showing left parietal intraparenchymal hemorrhage and SAH within left cerebral sulci.
B: Axial FLAIR redemonstrating the intraparenchymal hematoma and SAH, as well as small left
convexity subdural hematoma. C: Axial T2 weighted-images confirms findings in B. D: Axial post
contrast T1 weighted-images do not show any underlying enhancing lesion.
D
Relative cerebral blood volume maps (rCBV) demonstrate
decreased perfusion related to the intraparenchymal hematoma
(orange arrow), as well as asymmetric perfusion in other areas,
secondary to vasoconstrictions (blue arrows).
Radiological findings
• RCVS can be complicated by intraparenchymal
hematoma with or without sulcal hemorrhage.
• Findings may raise suspicion for amyloid
angiopathy, underlying neoplasm or vascular
pathology, such as arteriovenous malformation
(AVM) or venous thrombosis.
• Amyloid angiopathy patients are usually older
males with brain imaging showing hemorrhages
of different ages.
• Advanced and angiographic images may be
necessary to exclude underlying neoplasm or
vascular pathology, respectively.
Management
• No specific guideline as no trial in the literature.
• Avoiding inciting factor if able to identify.
• Close monitoring for early detection of possible
complications.
• Pain relief and supportive measures for
associated symptoms.
• Calcium-channel block to revert vasospasm.
• Intra-arterial vasodilators in patients with major
complications.
• Angioplasty as last resort in refractory cases.
Summary
• Increased awareness about different imaging
presentations of RCVS is desired to direct
appropriate and timely clinical management.
• Atypical subarachnoid hemorrhage, unexplained
cerebral edema and hemorrhage, as well as single
or multivessel diffuse narrowing with a relatively
normal appearing brain should raise suspicion of
RCVS.
• Patients with a typical presentation and normal
imaging findings should be presumed RCVS to have
until follow up vascular imaging is performed.
References
1.
2.
3.
4.
5.
6.
Marder CP, Donohue MM, Weinstein JR, Fink KR. Multimodal imaging of
reversible cerebral vasoconstriction syndrome: a series of 6 cases. AJNR Am J
Neuroradiol. 2012 Aug;33(7):1403-10.
Mehdi A, Hajj-Ali RA. Reversible cerebral vasoconstriction syndrome: a
comprehensive update. Curr Pain Headache Rep. 2014 Sep;18(9):443.
Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review:
reversible cerebral vasoconstriction syndromes. Ann Intern Med.
2007;146(1):34–44.
Miller TR, Shivashankar R, Mossa-Basha M, Gandhi D. Reversible Cerebral
Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical
Course. AJNR Am J Neuroradiol. 2015 Jan 15.
Miller TR, Shivashankar R, Mossa-Basha M, Gandhi D. Reversible Cerebral
Vasoconstriction Syndrome, Part 2: Diagnostic Work-Up, Imaging Evaluation,
and Differential Diagnosis. AJNR Am J Neuroradiol. 2015 Jan 22.
Papathanasiou A, Zouvelou V, Breen DP, Phillips TJ, Misbahuddin A, Chawda
S, de Silva R. Reversible cerebral vasoconstriction syndrome as a cause of
thunderclap headache: a retrospective case series study. Am J Emerg Med.
2014 Dec 19. pii: S0735-6757(14)00928-0.
References
7.
Kameda T, Namekawa M, Shimazaki H, Minakata D, Matsuura T,
Nakano I. Unique combination of hyperintense vessel sign on initial
FLAIR and delayed vasoconstriction on MRA in reversible cerebral
vasoconstriction syndrome: a case report. Cephalalgia. 2014
Nov;34(13):1093-6.
8. John S, Hajj-Ali RA. CNS Vasculitis. Semin Neurol 2014;34:405–412.
9. Sheikh HU, Mathew PG. Reversible cerebral vasoconstriction
syndrome: updates and new perspectives. Curr Pain Headache Rep.
2014 May;18(5):414.
10. Ioannidis I, Nasis N, Agianniotaki A, Katsouda E, Andreou A.
Reversible cerebral vasoconstriction syndrome: treatment with
multiple sessions of intra-arterial nimodipine and angioplasty. Interv
Neuroradiol. 2012 Sep;18(3):297-302.
11. Calic Z, Cappelen-Smith C, Zagami AS. The Reversible Cerebral
Vasoconstriction Syndrome. Intern Med J. 2014 Dec 16.
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