321 lines
30 KiB
Markdown
321 lines
30 KiB
Markdown
---
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title: "Multifocal Arterial Narrowing"
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docid: "761665cd-65e1-48e7-a539-1a5d7c148a6b"
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authors:
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- key: "2bca6b86-1eca-4e93-b997-4e18913686a7"
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value: "Hediyeh Baradaran, MD, MS"
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- key: "f6dcad15-b660-4243-a242-a9845c929101"
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value: "J. Scott McNally, MD, PhD"
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breadcrumbs:
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-
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name: "Brain"
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slug: "brain"
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treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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-
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name: "Differential Diagnosis"
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slug: "differential-diagnosis"
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treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
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-
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name: "Arteries"
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slug: "arteries"
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treeNodeId: "f5fee8ee-062f-41f2-b449-efcca9fb78f9"
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-
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name: "Anatomically Based Differentials"
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slug: "anatomically-based-differentials"
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treeNodeId: "9955b05e-9840-401a-b3da-4039f8b22a35"
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-
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name: "Multifocal Arterial Narrowing"
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slug: "multifocal-arterial-narrowing"
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treeNodeId: null
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category: "Brain"
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documentVersionId: "c67290b9-b36e-40c7-8b42-406b9bfad39e"
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imageCount: 25
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lastUpdated: "02/22/23"
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pageDescription: "Multifocal Arterial Narrowing"
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pageKeywords: "Brain, Differential Diagnosis, Arteries, Anatomically Based Differentials, Multifocal Arterial Narrowing"
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pageTitle: "Multifocal Arterial Narrowing | STATdx"
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enhancedTitle: "Multifocal Arterial Narrowing"
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type: "DDX"
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references: true
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breadcrumbs:
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- "Brain"
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- "Differential Diagnosis"
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- "Arteries"
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- "Anatomically Based Differentials"
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- "Multifocal Arterial Narrowing"
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---
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# ESSENTIAL INFORMATION
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- ## Key Differential Diagnosis Issues
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- **Clinical history major determinant of DDx (if available)**
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- **Arteriosclerotic vascular disease**(ASVD): Clinical history of uncontrolled diabetes, hypertension, hyperlipidemia, smoking
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- **Reversible cerebral vasoconstriction syndrome**(RCVS): Thunderclap headache and vasoactive drug use (cannabis, SSRI)
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- **Vasospasm**: Recent subarachnoid hemorrhage (SAH)
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- **Vasculitis**: Smoldering headache and CSF/systemic inflammatory markers
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- **Intravascular large B-cell lymphoma (IVL):** DDx excluded, progression despite treatment
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- **Effect of patient age on DDx**
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- **Middle-aged or older adults**
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- ASVD, vasculitis, RCVS
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- **Child or young adult**
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- Transient arteriopathy of young, moyamoya, inherited vasculopathy (e.g., Ehlers-Danlos), congenital infection (e.g., HIV vasculopathy)
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- **Is there hemorrhage?**
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- **Subarachnoid**→ RCVS, vasospasm, dissection, or pseudoaneurysm
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- **Parenchymal**→ vasculitis, RCVS, moyamoya, IVL
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- ## Helpful Clues for Common Diagnoses
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- **Atherosclerosis****, Intracranial**
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- **Location**
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- ASVD occurs in areas of flow reversal and oscillatory shear stress (e.g., bifurcations)
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- Proximal circle of Willis branches, vertebrobasilar, internal carotid arteries (ICAs)
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- **Lumen imaging of intracranial atherosclerosis**
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- Focal stenoses, lumen irregularities, elongation/ectasia
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- Stenosis measured by WASID criteria
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- CTA vs. 3D time-of-flight (TOF) MRA
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- CTA has slightly higher resolution than 3D TOF
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- 3D TOF can be limited in tortuous vessels with signal loss in inferiorly directed branches
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- 3D TOF MRA at 3T outperforms CTA in distal branches due to venous contamination
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- 3D TOF MIPs outperform CTA MIPs
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- **MR + CT**
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- High T2 signal → lipid/necrotic core (ASVD)
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- High T1 signal → intraplaque hemorrhage (less common)
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- Enhancement → unstable/active ASVD
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- Highly associated with acute territorial stroke; can persist for months
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- High repeat stroke risk despite medical therapy (15-30% per year)
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- Positive remodeling → outward bulging of vessel wall as compensatory response to plaque; may have little vessel narrowing on angiographic imaging
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- Calcification on CT/CTA → atherosclerosis
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- Most common cause of vessel narrowing ASVD, **not**vasculitis
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- ## Helpful Clues for Less Common Diagnoses
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- **Reversible Cerebral Vasoconstriction Syndrome**
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- **Etiology**
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- Spontaneous, related to vasoactive substances, postpartum state
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- **Lumen imaging**
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- Multifocal segmental narrowing, ± dilatations
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- **Vessel wall imaging**
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- No T2 signal, no or mild enhancement
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- **Vasospasm**
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- **Etiology**
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- Most common: Aneurysmal SAH
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- Less common: Trauma
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- **Lumen imaging**
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- Multifocal narrowing
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- **Vessel wall imaging**
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- No T2 signal, no or mild enhancement
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- **Vasculitis**
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- **Etiology**
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- Primary arteritis of CNS
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- Secondary vasculitis
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- Infectious
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- Autoimmune
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- **Lumen imaging**
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- Multifocal alternating stenoses, dilatations
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- **Vessel wall imaging**
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- T2 signal: None or mild
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- Wall enhancement: Positive, concentric
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- **Vasculopathy, Non-ASVD**
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- **Lumen imaging**
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- Fusiform or alternating areas of stenosis and dilatation, "beading"
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- Long, nonbranching vessel segments
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- **Vessel wall imaging**
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- Not well studied, likely active and inactive stages similar to ASVD
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- Younger patients
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- Vertebrobasilar > carotid
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- Inherited (e.g., Ehlers-Danlos) or acquired (e.g., viral)
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- **Dissection**
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- **Pathophysiology**
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- Can be traumatic or spontaneous, ± SAH
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- Often combined with pseudoaneurysm (dissecting pseudoaneurysm)
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- **Location**
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- Vertebral > > ICA
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- **Lumen imaging**
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- Lumen irregularity, ± dilation (pseudoaneurysm)
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- Reimage if dilation, risk of growth/rupture
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- **Vessel wall imaging**
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- T2 signal: Variable high signal, depends on age
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- T1 signal: High signal if subacute (~ 2-8 weeks old)
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- Wall enhancement: Avid if acute
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- **Pseudoaneurysm**
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- **Pathophysiology**
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- Lacks normal arterial wall layers, contained by adventitia or cavitated clot
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- Trauma, infection = common causes
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- Rapidly changes within hours or days, easily ruptures
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- **Location**
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- Can be peripheral location (distal to circle of Willis)
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- Often adjacent to skull base fractures or dura
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- e.g., dorsal variant ICA blister aneurysm
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- **Lumen imaging**
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- Often adjacent vessel segment irregular
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- Suspect pseudoaneurysm if broad-based bulge without neck
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- Look carefully for pseudoaneurysms in "angiogram-negative" SAH
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- ## Helpful Clues for Rare Diagnoses
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- **Intravascular Large B-Cell Lymphoma**
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- Consider after excluding other diagnoses
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- Often initial response with steroids with recrudescence of symptoms and progression of infarcts and hemorrhages
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- **Moyamoya**
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- Characteristic "puff of smoke" on DSA
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- Most often seen in children and young adults
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- **Radiation Vasculopathy**
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- Consider radiation vasculopathy if age-accelerated vessel narrowing/ASVD
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- History of prior radiation key
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- ## Other Essential Information
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- **Clinical management of intracranial atherosclerosis**
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- SAMPRIS trial: Intensive medical therapy superior to stenting
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- Recommended by SAMPRIS for symptomatic intracranial ASVD
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- Life coach, smoking cessation, dual antiplatelets, antihypertensive therapy, statins
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- Still had ~ 15% annual stroke risk
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- **Artifacts and limitations**
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- Motion degrades CTA, MRA, and vessel wall MR
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- Motion affects studies with longer acquisition times: Vessel wall MR > MRA > CTA
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- Motion degrades black blood vessel wall MR due to misregistration artifact ("ghosting" can mimic wall enhancement)
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- Pulsation artifact
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- Can obscure vessel narrowing on 3D TOF
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- Occurs in phase-encoding direction
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- Occluded vessels difficult to detect on CTA/MRA
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- T2 MR to troubleshoot (e.g., T2 SPACE)
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- CSF pulsation artifact may cause spin dephasing and mimic pathology (especially around distal basilar artery)
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- DANTE prepulse eliminates CSF pulsation artifact
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- Incomplete suppression of flowing contrast in black blood imaging
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- DANTE prepulse eliminates flowing contrast
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- DANTE T1 SPACE much higher signal:noise ratio and contrast:noise ratio than T1 SPACE alone
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- Slow-flow and entry slice phenomenon → T1 shortening may mimic thrombus
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- Venous contrast on CTA limits distal branch evaluation (3rd and 4th order)
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- 3D TOF MRA at 3T outperforms CTA in these areas
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## References
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# Selected References
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1. [Culleton S et al: MRI detection of carotid intraplaque hemorrhage and postintervention cognition. AJNR Am J Neuroradiol. 43(12):1762-9, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36357151%5Bpmid%5D)
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1. [Larson AS et al: Nonstenotic carotid plaques and embolic stroke of undetermined source: a multimodality review. AJNR Am J Neuroradiol. ePub, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36549844%5Bpmid%5D)
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1. [McCarty JL et al: Ischemic infarction in young adults: a review for radiologists. Radiographics. 39(6):1629-48, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31589580%5Bpmid%5D)
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1. [Schaafsma JD et al: Diagnostic Impact of intracranial vessel wall MRI in 205 patients with ischemic stroke or TIA. AJNR Am J Neuroradiol. 40(10):1701-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31488500%5Bpmid%5D)
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1. [Mandell DM et al: Intracranial vessel wall MRI: principles and expert consensus recommendations of the American Society of Neuroradiology. AJNR Am J Neuroradiol. 38(2):218-29, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27469212%5Bpmid%5D)
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1. [Alexander MD et al: High-resolution intracranial vessel wall imaging: imaging beyond the lumen. J Neurol Neurosurg Psychiatry. 87(6):589-97, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26746187%5Bpmid%5D)
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1. [Lehman VT et al: Clinical interpretation of high-resolution vessel wall MRI of intracranial arterial diseases. Br J Radiol. 89(1067):20160496, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27585640%5Bpmid%5D)
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1. [Mossa-Basha M et al: Added value of vessel wall magnetic resonance imaging in the differentiation of moyamoya vasculopathies in a non-Asian Cohort. Stroke. 47(7):1782-8, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27272486%5Bpmid%5D)
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1. [Miller TR et al: Reversible cerebral vasoconstriction syndrome, part 1: epidemiology, pathogenesis, and clinical course. AJNR Am J Neuroradiol. 36(8):1392-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25593203%5Bpmid%5D)
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1. [Miller TR et al: Reversible cerebral vasoconstriction syndrome, part 2: diagnostic work-up, imaging evaluation, and differential diagnosis. AJNR Am J Neuroradiol. 36(9):1580-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25614476%5Bpmid%5D)
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1. [Mossa-Basha M et al: Multicontrast high-resolution vessel wall magnetic resonance imaging and its value in differentiating intracranial vasculopathic processes. Stroke. 46(6):1567-73, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25953365%5Bpmid%5D)
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## Images
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### Selected Images
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**Atherosclerosis, Intracranial**
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*MIP MRA shows multifocal stenoses <img src='img/arrows/CS.png'/>, characteristic for intracranial atherosclerosis, the most common cause of alternating stenoses and dilatations. Risk factors include diabetes, hypertension, hyperlipidemia, and smoking.*
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**Atherosclerosis, Intracranial**
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*MIP MRA shows multifocal stenoses <img src='img/arrows/CS.png'/>, characteristic for intracranial atherosclerosis, the most common cause of alternating stenoses and dilatations. Risk factors include diabetes, hypertension, hyperlipidemia, and smoking.*
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**Atherosclerosis, Intracranial**
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*CTA in the right middle cerebral artery (MCA) shows focal severe stenosis <img src='img/arrows/CS.png'/> with associated avid enhancement <img src='img/arrows/WS.png'/> on postcontrast vessel wall MR as well as T2 hyperintensity <img src='img/arrows/BS.png'/> in the vessel wall, consistent with active atherosclerotic plaque.*
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**Reversible Cerebral Vasoconstriction Syndrome**
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*NECT in a patient with RCVS shows vertex subarachnoid hemorrhage (SAH) <img src='img/arrows/WS.png'/>. There is no associated enhancement on MR T1 DANTE SPACE pre- <img src='img/arrows/CS.png'/> or post- <img src='img/arrows/CO.png'/> contrast. Typical presentation includes a thunderclap headache; history of vasoactive drug use is also common.*
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**Reversible Cerebral Vasoconstriction Syndrome**
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*DSA in the same patient shows multifocal narrowing <img src='img/arrows/BS.png'/> on the initial ICA injection. Following Verapamil injection, there is reversal of the multifocal narrowing <img src='img/arrows/CS.png'/> with a more normal caliber of the intracranial vasculature, compatible with RCVS.*
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**Vasospasm**
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*Lateral DSA shows a saccular aneurysm <img src='img/arrows/BO.png'/> and narrowed cortical vessels <img src='img/arrows/BS.png'/>, indicating a vasospasm caused by an aneurysmal SAH (aSAH).*
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**Vasospasm**
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*DWI MR in a traumatic brain injury patient shows SAH and right MCA vasospasm with acute infarct <img src='img/arrows/WS.png'/>. The right MCA proximal M2 is narrowed on 3D TOF MR <img src='img/arrows/WO.png'/>. No intrinsic MR T1 signal is seen on precontrast <img src='img/arrows/CS.png'/> image, and there is no enhancement on postcontrast <img src='img/arrows/CO.png'/> DANTE T1 SPACE, consistent with posttraumatic SAH-induced vasospasm.*
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**Vasculitis**
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*3D TOF MRA in a young patient with vasculitis and MCA stroke shows lumen irregularity of the right anterior circulation <img src='img/arrows/WS.png'/>. DSA confirms lumen findings <img src='img/arrows/BO.png'/> and more subtle right M2 MCA narrowing <img src='img/arrows/WO.png'/>. Avid wall enhancement is on DANTE T1 SPACE comparing pre- <img src='img/arrows/WC.png'/> (magnified <img src='img/arrows/CS.png'/>), vs. postcontrast <img src='img/arrows/CC.png'/> (magnified <img src='img/arrows/CO.png'/>).*
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**Vasculopathy, Non-ASVD**
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*MIP MRA in a drug user shows multifocal narrowing in the right MCA and anterior cerebral artery branches <img src='img/arrows/WS.png'/>, confirmed on DSA <img src='img/arrows/CS.png'/>, compatible with drug-induced vasculopathy.*
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**Dissection**
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*Axial T1 C+ FS MR in a patient with posterior circulation ischemic symptoms after severe deceleration injury shows both vertebral arteries markedly enlarged by subacute clot <img src='img/arrows/WS.png'/>. The right vertebral artery is completely thrombosed, while small residual lumen is seen on the left <img src='img/arrows/WO.png'/>.*
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**Pseudoaneurysm**
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*Right V4 vertebral artery pseudoaneurysm in a trauma patient with initial <img src='img/arrows/WS.png'/> and follow-up <img src='img/arrows/WO.png'/> 3D TOF MRA shows growth with 2 areas of dilation <img src='img/arrows/CO.png'/>, confirmed on angiography (DSA <img src='img/arrows/BS.png'/> and 3D <img src='img/arrows/CS.png'/>).*
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**Pseudoaneurysm**
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*Dorsal variant ICA pseudoaneurysm is shown on 3D TOF MRA <img src='img/arrows/WS.png'/> and DSA <img src='img/arrows/BO.png'/> with wall enhancement on MR T1 SPACE <img src='img/arrows/WO.png'/> and magnified views in bottom right.*
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**Intravascular Large B-Cell Lymphoma**
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*Biopsy-proven intravascular lymphoma with multifocal hemorrhages, infarcts, and surrounding edema <img src='img/arrows/WS.png'/> is shown on FLAIR, DTI, and SWI. DSA shows multifocal distal vessel lumen irregularity <img src='img/arrows/CS.png'/>. Vessel wall MR was negative. Intravascular large B-cell lymphoma is often a diagnosis of exclusion. Skin biopsy or brain biopsy is typically needed to make the diagnosis.*
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**Moyamoya**
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*Lateral DSA angiography shows tapered occlusion <img src='img/arrows/BS.png'/> of supraclinoid ICA with a tangle of "puff of smoke" lenticulostriate <img src='img/arrows/BO.png'/>, characteristic of moyamoya.*
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**Radiation Vasculopathy**
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*Axial DWI shows a recent infarct <img src='img/arrows/WS.png'/> superimposed on a FLAIR hyperintense <img src='img/arrows/BS.png'/>, remote right posterior cerebral artery (PCA) infarct with delayed MTT <img src='img/arrows/WO.png'/> in a patient with radiation vasculopathy. DSA shows severe right PCA narrowing with near occlusion <img src='img/arrows/CS.png'/> and contralateral PCA narrowing <img src='img/arrows/CO.png'/>.*
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### Additional Images
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**Vasculopathy, Non-ASVD**
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*Lateral angiography shows a posterior communicating ICA aneurysm with multiple lobulations <img src='img/arrows/BS.png'/> and an unusually long "aspect ratio." This atypical aneurysm is likely related to a vasculopathy.*
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**Pseudoaneurysm**
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*Lateral angiography in a patient with a skull base fracture and stroke shows a pseudoaneurysm <img src='img/arrows/BS.png'/> at the junction of the ascending/horizontal cavernous carotid artery. Note narrowing <img src='img/arrows/BO.png'/> and intimal irregularity <img src='img/arrows/BC.png'/>, a potential source for embolic stroke.*
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**Pseudoaneurysm**
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*Axial T2 MR in a 30-year-old man with headache, a history of a motor vehicle accident and basilar skull fracture 10 years earlier shows a cavernous pseudoaneurysm <img src='img/arrows/WO.png'/>.*
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**Pseudoaneurysm**
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*Anteroposterior angiography in a patient who decompensated clinically 24 hours after admission for a closed head injury shows traumatic pseudoaneurysm <img src='img/arrows/BO.png'/> caused by impingement of ACA against falx.*
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**Atherosclerosis, Intracranial**
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*3D TOF MPR <img src='img/arrows/CO.png'/> and axial TOF <img src='img/arrows/WS.png'/> show ~ 50% narrowing of the left V4 vertebral artery. T2 SPACE shows a lipid/necrotic core <img src='img/arrows/BS.png'/>. MPRAGE is positive for IPH <img src='img/arrows/CS.png'/>. DANTE T1 SPACE pre- <img src='img/arrows/WC.png'/> and post- <img src='img/arrows/WO.png'/> contrast images demonstrate avid wall enhancement (active plaque).*
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**Vasculopathy, Non-ASVD**
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*HIV vasculopathy with bilateral fusiform aneurysms of the terminal ICAs is shown. A T2-hyperintense clot is present in the right ICA terminus aneurysm <img src='img/arrows/WS.png'/>, and there is a preserved flow void in the aneurysmal left ICA terminus <img src='img/arrows/CS.png'/>. CTA shows an occluded right terminal ICA aneurysm <img src='img/arrows/WO.png'/> and patent left terminal ICA fusiform aneurysm <img src='img/arrows/WC.png'/>. DSA confirms these findings with a fusiform aneurysm of the left ICA terminus <img src='img/arrows/CO.png'/>.*
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**Reversible Cerebral Vasoconstriction Syndrome**
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*Postpartum RCVS shows slow flow/vertex SAH on FLAIR <img src='img/arrows/WS.png'/>, multivessel narrowing on 3D TOF <img src='img/arrows/WC.png'/>, and absent enhancement on DANTE T1 SPACE pre- <img src='img/arrows/CS.png'/> or post- <img src='img/arrows/CO.png'/> contrast.*
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**Atherosclerosis, Intracranial**
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*Anteroposterior angiography shows multifocal stenoses characteristic for atherosclerosis, the most common cause of alternating stenoses and dilatations.*
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**Atherosclerosis, Intracranial**
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*DSA shows multifocal stenoses <img src='img/arrows/CS.png'/> characteristic for atherosclerosis, the most common cause of alternating stenoses and dilatations.*
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**Vasculopathy, Non-ASVD**
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*DSA shows fusiform elongation of an MCA branch <img src='img/arrows/BO.png'/>. The patient later admitted to using street drugs.*
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**Pseudoaneurysm**
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*Lateral angiography shows a classic blood, blister-like aneurysm <img src='img/arrows/BS.png'/> along the greater curvature of the supraclinoid ICA. A hemispherical bulge with a broad orifice is typical for blood blister aneurysms. (Courtesy D. Phillips, MD).*
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