Prevalence of and risk factors for enlarged perivascular spaces in adult patients with moyamoya disease

Abstract

BackgroundEnlarged perivascular spaces (EPVS) are often observed with magnetic resonance imaging in patients with small vessel disease. However, the risk factors, radiological features, and clinical relevance of EPVS in patients with moyamoya disease are poorly understood. The purpose of this study was to evaluate EPVS, the risk factors of many EPVS, and the pathophysiology of EPVS in adult patients with moyamoya disease.MethodsOne hundred cerebral hemispheres of 50 adult patients with moyamoya disease were examined. The control group consisted of 50 age/sex-matched patients without ischemic disease. The numbers of EPVS at the level of the centrum semiovale per hemisphere were compared between the moyamoya disease and control groups. In each hemisphere, the total numbers of EPVS were categorized into five grades (0–4), and the clinical and radiological characteristics of the predictive factors in patients in the high EPVS grade group (EPVS grade = 4) were assessed.ResultsThe EPVS counts and grades were significantly higher in the moyamoya disease group. Analyses of the background characteristics of the patients with moyamoya disease revealed that significantly higher prevalence of high EPVS grades were associated with the female sex, hypertension, high magnetic resonance angiography scores, high numbers of flow voids in the basal ganglia, high brain atrophy scores, ivy signs, and white matter lesions. A logistic multivariate analysis of the patients with high EPVS grades revealed significant associations with the female sex, hypertension, and flow voids in the basal ganglia.ConclusionsIncreased EPVS were confirmed in adult patients with moyamoya disease, and the associated clinical and radiological factors were identified. The presence of hypertension, the female sex, and flow voids in the basal ganglia were important for predicting high EPVS grades in patients with moyamoya disease. Reductions in arterial pulsations with steno-occlusive changes can inhibit the flow of interstitial fluid, which can increase the number of EPVS in patients with moyamoya disease. Other clinical factors, such as the female sex and hypertension, may promote secondary brain damage in patients with moyamoya disease. Further evaluations of EPVS in patients with moyamoya disease are needed to better understand their pathophysiological importance.

<p>The relationship between blood–brain barrier permeability and enlarged perivascular spaces: a cross-sectional study</p>

“…We found that participants with high-grade BG-EPVS had a lower fractional blood plasma volume. As blood plasma volume is associated with the cerebral blood flow (CBF), this observation was in line with the result of a previous study30 suggesting that patients with high-grade EPVS were significantly associated with Moyamoya disease which features lower CBF and reduced cerebral perfusion pressure. This may suggest that hypoperfusion is involved in the pathogenesis of EPVS.…”

Abnormalities in spinal cord ultrastructure in a rat model of post-traumatic syringomyelia

“…2) reported in this study are consistent with earlier reports on the ultrastructure of human and rabbit syrinx cavities [14,15,32,33]. Interestingly, enlarged perivascular spaces were also reported in hydrocephalus [34][35][36] and a number of cerebrovascular and systemic pathologies including small vessel disease [37,38], moyamoya disease [39], ischemic and lacunar stroke [40], lupus erythematosus [41], and arteriosclerosis [42]. Despite frequent reports [34][35][36][37][38][39][40][41][42], the significance of enlarged perivascular spaces remains unclear.…”

“…Interestingly, enlarged perivascular spaces were also reported in hydrocephalus [34][35][36] and a number of cerebrovascular and systemic pathologies including small vessel disease [37,38], moyamoya disease [39], ischemic and lacunar stroke [40], lupus erythematosus [41], and arteriosclerosis [42]. Despite frequent reports [34][35][36][37][38][39][40][41][42], the significance of enlarged perivascular spaces remains unclear. There is a possibility that blood vessels with enlarged perivascular spaces are initially involved in the process of syrinx formation, however there was no explicit evidence of enlarged perivascular spaces directly feeding into the cavities in this study.…”

Increased parenchymal free water may be decreased by revascularization surgery in patients with moyamoya disease

“…This result is consistent with a previous study using NODDI and showed increased extracellular free water (V iso ) in patients with MMD [14]. Previous studies reported increased perivascular spaces in patients with moyamoya disease [23,24] and suggested that the increased free water may result from an impaired glymphatic system that uses arterial input as the driving force [25,26]. Disruption of the glymphatic system is also suggested in some studies evaluating acute ischemic stroke [27].…”