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The diagnostic contribution of intracranial vessel wall imaging in the differentiation of primary angiitis of the central nervous system from other intracranial vasculopathies

  • Diagnostic Neuroradiology
  • Published:
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Abstract

Purpose

The aim of this study is to demonstrate the diagnostic effect of VWI in differentiating PACNS from other vasculopathies and its role in post-treatment follow-up in PACNS patients in this study.

Methods

In this prospective study, we included patients with clinical suspicion of PACNS who presented with new-onset ischemic events and had significant intracranial large vessel stenosis on DSA or MRA. VWI was performed on all patients. The imaging findings and final diagnoses were recorded. Control VWI was performed on patients with PACNS diagnosis after at least 3 months of treatment, and the change in findings was also evaluated.

Results

Twenty-three patients were included in the study had a median age of 40 (range 12–58). The most common clinical manifestations were focal neurologic deficits. According to the initial clinical evaluation, 10 patients (43.5%) were classified as PACNS and 13 patients (56.5%) as indeterminate for PACNS. After incorporating the VWI findings, the diagnosis of PACNS was confirmed in all clinically diagnosed PACNS patients. Concentric wall thickening and contrast enhancement were statistically significant in the PACNS group (p <0.001). According to concentric thickening and VWE features, sensitivity and specificity in distinguishing PACNS and other vasculopathies were 95.2%, 75% and 95.2%, 68.8%, respectively. Vessel wall enhancement regressed in 7 of 9 patients during a median follow-up period of 8 months (range 5.5–11.5) in PACNS patients who followed up.

Conclusion

VWI seems a new and useful imaging method in the differential diagnosis of PACNS and might be a useful adjunct for post-treatment follow-up.

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Data availability

Not applicable.

Code availability

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Abbreviations

VW-MRI:

Vessel wall magnetic resonance imaging

PACNS:

Primary angiitis of the central nervous system

RCVS:

Reversible cerebral vasoconstriction syndrome

MMD:

Moya Moya disease

VWE:

Vessel wall enhancement

VWT:

Vessel wall thickening

VWI:

Vessel wall imaging

CNS:

Central nervous system

CSF:

Cerebrospinal fluid

TIA:

Transient ischemic attack

DSA:

Digital subtraction angiography

DWI:

Diffusion-weighted imaging

SWI:

Susceptibility-weighted imaging

TOF-MRA:

Time of flight MR angiography

MIP:

Maximum intensity projection

ACA:

Anterior cerebral artery

MCA:

Middle cerebral artery

PCA:

Posterior cerebral artery

ICA:

Internal carotid artery

CSF:

Cerebrospinal fluid

F/U:

Follow-up

S:

Steroid

MTX:

Methotrexate

Aza:

Azathioprine

Cfc:

Cyclophosphamide

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Funding

No funding was received for conducting this study.

Author information

Authors and Affiliations

  1. Department of Radiology, Sureyyapasa Chest Diseases and Thoracic Surgery Training Hospital, Başıbüyük Mah. Hastane Yolu Cad, 34844, Istanbul, Turkey

    Ahmet Kursat Karaman

  2. Department of Radiology, Division of Neuroradiology, Istanbul University-Cerrahpasa, Istanbul, Turkey

    Bora Korkmazer, Serdar Arslan, Osman Kızılkılıc, Naci Kocer & Civan Islak

  3. Department of Neurology, Istanbul University-Cerrahpasa, Istanbul, Turkey

    Ugur Uygunoglu

  4. Department of Radiology, School of Medicine, Acibadem Mehmet Ali Aydınlar University, Istanbul, Turkey

    Ercan Karaarslan

Authors
  1. Ahmet Kursat Karaman
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Contributions

Conception and design: Civan Islak, Naci Kocer, and Ahmet Kursat Karaman. Data collection, data analysis: Ahmet Kursat Karaman, Ugur Uygunoglu, Bora Korkmazer, and Ercan Karaarslan. Drafting the article: Ahmet Kursat Karaman, Bora Korkmazer, Serdar Arslan, Osman Kızılkılıc, and Ercan Karaarslan. Critical revision of the manuscript: Serdar Arslan, Ugur Uygunoglu, Osman Kızılkılıc, Naci Kocer, and Civan Islak. All listed authors have approved the manuscript before submission, including the names and order of authors; managing all communication between the Journal and all co-authors, before and after publication; * providing transparency on the re-use of material and mention any unpublished material included in the manuscript in a cover letter to the Editor; making sure disclosures, declarations, and transparency on data statements from all authors are included in the manuscript as appropriate.

Corresponding author

Correspondence to Ahmet Kursat Karaman.

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The authors declare no relevant conflicts of interest.

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All procedures performed in the studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Karaman, A.K., Korkmazer, B., Arslan, S. et al. The diagnostic contribution of intracranial vessel wall imaging in the differentiation of primary angiitis of the central nervous system from other intracranial vasculopathies. Neuroradiology 63, 1635–1644 (2021). https://doi.org/10.1007/s00234-021-02686-y

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