Contrast enhancement patterns associated with acute stroke in moyamoya disease using MR vessel wall imaging.

BACKGROUND AND PURPOSE: High-resolution magnetic resonance imaging (HR-MRI) can provide valuable insights into the histopathological characteristics of moyamoya disease (MMD). However, the patterns of vessel wall contrast enhancement have not been well established. We aimed to identify the contrast enhancement patterns of the vessel walls associated with acute cerebral infarction using HR-MRI in MMD. METHODS: In this retrospective study, we conducted genetic tests for Ring Finger Protein 213 (RNF 213) and performed HR-MRI on patients suspected of having MMD. We analyzed wall enhancement patterns including concentric, eccentric, or mixed enhancement types, and the occurrence of acute cerebral infarction in patients who simultaneously tested positive for RNF 213 and exhibited definite features of MMD on HR-MRI. RESULTS: Among 306 patients who underwent RNF 213 tests for the evaluation of MMD, 56 showed positive RNF 213, and HR-MRI was performed on 32 of them. Among the patients with acute cerebral infarction, the incidence rate was significantly higher in the group with concentric wall enhancement compared to patients without acute cerebral infarction (73.3% vs. 17.0%, p < .002). Furthermore, the incidence was notably elevated, even in patients with pure concentric wall enhancement (40.0% vs. 5.9%, p = .033). The area under the curve (AUC) for the group with any concentric wall enhancement showed a significant result of .78 (95% confidence interval [CI]: .61-.95, p = .007), whereas the predictive ability for pure concentric wall enhancement did not reach significance (AUC = .67, 95% CI: .48-.86, p = .100). CONCLUSIONS: Concentric wall enhancement was a significant predictor of acute cerebral infarction in patients with MMD.

Approach to adult Moyamoya disease presenting as bilateral anterior circulation infarcts: a case report.

Moyamoya disease (MMD) is a rare progressive condition of unknown etiology that affects the blood vessels in the brain and is characterized by narrowing or stenosis of the distal parts of the internal carotid and major intracranial arteries, leading to the development of fragile collateral supplies. This makes it a significant causative factor for both ischemic strokes as well as intracerebral hemorrhages. As per the recommendations by the Research Committee on Moyamoya disease in 2021, the diagnostic criteria for MMD are essentially based on indicative radiological findings on cerebral angiography or MRI Brain and MRA, after excluding possible differentials. In this case report, we present the case of a 45-year-old Asian lady who presented with sudden-onset headaches and right-sided weakness. Her admission non-contrast CT brain scan was undiagnostic. MRI brain and MRA scans revealed bilateral anterior circulation watershed infarcts of variable age along with supra-clinoid stenosis. She was initially treated with steroids for vasculitis after discussions with a multidisciplinary team involving neurologists, stroke physicians and neuro-radiologists; however, further imaging revealed a diagnosis of MMD, and she was subsequently referred for revascularization surgery. Given the limited number of similarly documented cases, we hope this report will contribute to the knowledge base.