Arterial wall contrast enhancement in progressive moyamoya disease

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  • 1 Department of Neurosurgery and Center for Moyamoya and Cerebral Revascularization and
  • 2 Department of Diagnostic and Interventional Neuroradiology, University of Tübingen, Tübingen, Germany; and
  • 3 Moyamoya Center, Division of Pediatric Neurosurgery, University Children’s Hospital Zürich, Zürich, Switzerland
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OBJECTIVE

The purpose of this study was to evaluate chronological patterns of arterial wall contrast enhancement in contrast-enhanced high-resolution MRI (CE-HR-MRI) in patients with moyamoya disease (MMD).

METHODS

The authors performed a blinded analysis of clinical and imaging data from MMD patients. Data were analyzed chronologically for each patient and the intensity of arterial wall enhancement was correlated with the clinical and imaging-based progression status of the disease.

RESULTS

A total of 31 MMD patients and 61 imaging time points were included. CE-HR-MRI results were available for 56 time points, representing 112 hemispheric analyses. No arterial wall contrast enhancement (grade 1) was seen in 54 (48%) of the analyses, mild enhancement (grade 2) in 24 (21%), moderate enhancement (grade 3) in 15 (13%), and strong (grade 4) mainly concentric arterial wall contrast enhancement in 19 (17%). Grade 4 contrast enhancement was significantly (p < 0.001) associated with clinical disease progression within 6 months (before or after the MRI) compared to grades 1–3, with positive and negative predictive values of 0.8 and 0.88, respectively. Grades 1 and 2 (no contrast enhancement and only mild contrast enhancement) were highly predictive for stable disease (negative predictive value: 0.95).

CONCLUSIONS

A specific chronological increasing and decreasing pattern of arterial wall contrast enhancement associated with “beginning” as well as progression of angiopathy occurs in MMD patients. In clinical practice, CE-HR-MRI of the arterial wall may help to identify patients at risk of new strokes caused by disease progression and hence impel early treatment for future stroke prevention. Understanding of this temporary enhancement of the arterial wall might also bring new insights into the etiology of MMD.

ABBREVIATIONS ACA = anterior cerebral artery; ACoA = anterior communicating artery; CE-HR-MRI = contrast-enhanced high-resolution MRI; DSA = digital subtraction angiography; ICA = internal carotid artery; MCA = middle cerebral artery; MMD = moyamoya disease; RI = remodeling index; TIA = transient ischemic attack.

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Contributor Notes

Correspondence Nadia Khan: Center for Moyamoya and Cerebral Revascularization, Tübingen, Germany, and Moyamoya Center, Children’s Hospital, University of Zürich, Switzerland. moyamoya@neurochirurgie-tuebingen.de.

INCLUDE WHEN CITING Published online May 24, 2019; DOI: 10.3171/2019.2.JNS19106.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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