Glymphatic impairment in Moyamoya disease

Abstract

Moyamoya disease (MMD) is a chronic disease characterized by the progressive narrowing of the terminal internal carotid artery, accompanied by abnormal angiogenesis at the base of the skull and defective formation of the vascular network, with a complex clinical picture and a risk of cognitive impairment and dementia in addition to ischemic and hemorrhagic events. The glymphatic system is a cerebrospinal fluid and interstitial fluid drainage pathway that acts throughout the brain to remove metabolic wastes from the brain parenchyma. Clinical studies have found that cognitive decline in patients with MMD is linked to metabolite accumulation and reduced diffusion tensor image analysis along the perivascular space (DTI-ALPS), highlighting the potential impact of glymphatic system impairment. This dysfunction may stem from a combination of chronic hypoperfusion, systemic microstructural damage and inflammatory response, and is an important link to further deterioration of vascular cognitive function. This article discusses the recent findings on glymphatic system disorders in MMD, with the objective of providing new approaches to the disease.

Keywords: Aquaporin 4; Cerebrospinal Fluid; Cognitive Impairment; Glymphatic System; Imaging Biomarkers; Metabolic Markers; Moyamoya Disease; Perivascular Space.