Disease of the blood vessels.
Accurate and timely diagnosis of intracranial vasculopathies is important due to significant risk of morbidity with delayed and/or incorrect diagnosis both from the disease process as well as inappropriate therapies. Conventional vascular imaging techniques for analysis of intracranial vascular disease provide limited information since they only identify changes to the vessel lumen. New advanced MR intracranial vessel wall imaging (IVW) techniques can allow direct characterisation of the vessel wall. These techniques can advance diagnostic accuracy and may potentially improve patient outcomes by better guided treatment decisions in comparison to previously available invasive and non-invasive techniques. While neuroradiological expertise is invaluable in accurate examination interpretation, clinician familiarity with the application and findings of the various vasculopathies on IVW can help guide diagnostic and therapeutic decision-making. This review article provides a brief overview of the technical aspects of IVW and discusses the IVW findings of various intracranial vasculopathies, differentiating characteristics and indications for when this technique can be beneficial in patient management 1).
Quantification of the severity of vasculopathy and its impact on parenchymal hemodynamics is a necessary prerequisite for informing management decisions and evaluating intervention response in patients with moyamoya disease.
Human immunodeficiency virus (HIV)-associated vasculopathy can cause ischemic stroke; however, there is limited evidence on optimal management.
Mizushima et al. reported a case of acute ischemic stroke due to progressive internal carotid artery stenosis in an HIV-positive patient. Superficial temporal artery to middle cerebral artery bypass), in addition to the best medical treatments, prevented stroke progression.
A 39-year-old man with HIV infection presented with a sudden onset of aphasia and right hemiparesis. Magnetic resonance imaging revealed an ischemic lesion in the leftbasal ganglia and concentric thickening of the vessel wall in the terminal portion of the bilateral ICAs. Despite maximal medical treatments for HIV-associated vasculopathy and possible opportunistic infections, bilateral ICA stenoses progressed, leading to a second hemodynamic stroke event. Because tissue plasminogen activator treatment failed, they performed STA-MCA bypass. A significant improvement in neurological symptoms and cerebral blood flow was observed after surgery. No further stroke events occurred during the continuation of medical treatments 2).
Primary vs. Secondary Vasculopathies:
Primary Vasculopathies: These are disorders in which the vascular abnormality is the primary condition. Examples include vasculitis, where inflammation damages blood vessel walls, and hereditary hemorrhagic telangiectasia (HHT), which involves abnormal development of blood vessels. Secondary Vasculopathies: These arise as a result of another underlying condition. For example, atherosclerosis is a common secondary vasculopathy where plaques build up in arteries due to factors like high cholesterol and inflammation. Vasculitis Classification:
Vasculitis refers to inflammation of blood vessels. It can be classified based on vessel size: Large-Vessel Vasculitis: Involves large arteries. Examples include giant cell arteritis and Takayasu arteritis. Medium-Vessel Vasculitis: Affects medium-sized arteries. Examples include polyarteritis nodosa and Kawasaki disease. Small-Vessel Vasculitis: Targets small arteries, arterioles, and capillaries. Examples include microscopic polyangiitis, granulomatosis with polyangiitis (formerly Wegener's granulomatosis), and Churg-Strauss syndrome. Hemorrhagic vs. Ischemic Vasculopathies:
Hemorrhagic Vasculopathies: Conditions characterized by bleeding due to vessel abnormalities. Cerebral cavernous malformations and hereditary hemorrhagic telangiectasia (HHT) fall into this category. Ischemic Vasculopathies: Conditions where blood flow to tissues is compromised, leading to ischemia. Atherosclerosis and thromboangiitis obliterans (Buerger's disease) are examples. Genetic vs. Acquired Vasculopathies:
Genetic Vasculopathies: Result from inherited genetic mutations. Examples include Ehlers-Danlos syndrome and Marfan syndrome, which affect connective tissues and blood vessels. Acquired Vasculopathies: Develop as a result of environmental factors, infections, autoimmune responses, or other non-genetic causes. Systemic vs. Organ-Specific Vasculopathies:
Systemic Vasculopathies: Affect blood vessels throughout the body. Vasculitis and systemic sclerosis are examples. Organ-Specific Vasculopathies: Primarily impact blood vessels in a specific organ or tissue. Retinopathy and nephropathy related to diabetes are organ-specific vasculopathies.