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Nearly half of all emergency department (ED) visits in the United States result in some type of imaging study, a large number of which are performed for neurological complaints. Common presenting neurological complaints include headache, weakness, stroke, and trauma. Often the initial study performed for the evaluation of these complaints is an unenhanced computed tomography (CT) of the brain. However, as will be discussed below, certain presentations and clinical history can warrant magnetic resonance imaging (MRI) as a superior first-line imaging test. This chapter seeks to provide guidance in imaging modality selection and to discuss imaging findings in common clinical presentations to the ED. When pertinent, alternative and recommended follow-up studies are also discussed
Vascular Ehlers-Danlos syndrome (vEDS) is a genetic disease that has an autosomal dominant pattern of inheritance and is caused by mutations in the COL3A1 gene, resulting in type III collagen synthesis defect, making blood vessels and organs particularly fragile and vulnerable. Vascular complications typically include arterial ruptures but also arterial dissections, which can cause ischaemia in the territory involved; visceral complications are also frequently reported. Patients may show a characteristic facial appearance; joint’s hypermobility and translucent skin with easy bruising are commonly found. Diagnostic criteria have been proposed to improve the diagnosis of this rare disorder, although the genetic confirmation is still required. There is no established treatment, so primary and secondary prevention play a key role in the management of these patients. For example, when a cerebrovascular event, such as a stroke, occurs as a result of a spontaneous dissection of a blood vessel, long-lasting antithrombotic therapy, which is the standard secondary prevention in stroke, is actually contraindicated, considering the high risk of bleeding associated with this disease
This chapter outlines some characteristics of vascular dementia (VaD) related to large-vessel diseases in terms of pathology, pathogenesis, and clinical aspects. Large-vessel VaD is characterized by a step-wise cognitive deterioration, focal signs and symptoms which are the result of repeated ischemic strokes. The chapter reviews some of large-vessel diseases such as atherosclerosis, arterial dissection, angiitis, moyamoya disease, and intracranial arterial dolichoectasia. The pathogenesis of large-vessel VaD is complex and to some extent remains a matter of investigation. Efforts in defining diagnostic criteria are made in order to characterize patterns of brain infarction from which it is reliable to deduce the presence of dementia. Some of the most important pathophysiological mechanisms and factors implicated in large-vessel VaD are volume of lesions, number of lesions, location of lesions, and coexistence of other brain pathologies, particularly Alzheimer's disease (AD).
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