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Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy

Key Points CADASIL is a familial small artery disease responsible for migraine with aura, stroke, disability, and cognitive decline. The disease occurs during midadulthood. CADASIL is caused by mutations of NOTCH3 gene. CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an inherited small artery disease of midadulthood that was identified using clinical tools (magnetic resonance imaging (MRI) and pathology) and genetic tools in…

Moyamoya Disease

Key Points Moyamoya disease is an unusual form of chronic cerebrovascular occlusive disease that is characterized by angiographic findings of bilateral stenosis or occlusion at the terminal portion of the internal carotid artery together with an abnormal vascular network at the base of the brain. The major cellular components of the thickened intima formed in arteries of the circle of Willis and the major branches are…

Stroke and Substance Abuse

Key Points Stroke in recreational substance users can be an indirect complication—for example, endocarditis and cardioembolism in parenteral drug users. With some drugs (e.g., cocaine), stroke appears to be the result of a direct effect. For illicit drugs, epidemiologic studies are few, but numerous case reports of stroke temporally associated with drug use in young subjects without other risk factors provide persuasive (albeit inconclusive) evidence for…

Posterior Reversible Encephalopathy Syndrome

Key Points Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiologic disorder characterized by rapidly progressive neurologic symptoms and a characteristic pattern of vasogenic edema on neuroimaging. Neurologic symptoms are usually associated with an increase in blood pressure above baseline. Malignant hypertension, preeclampsia/eclampsia, medications (especially calcineurin inhibitors), thrombotic microangiopathy syndromes, and sepsis are all associated with the development of PRES. Pathogenesis involves the breakdown of the blood-brain…

Reversible Cerebral Vasoconstriction Syndromes

Key Points Reversible cerebral vasoconstriction syndromes (RCVS) are a group of conditions characterized by reversible multifocal narrowing and dilatation of the cerebral arteries. The mean age is 42 years; age range 4 months to 76 years. The female:male ratio ranges from 2:1 to 10:1. The etiology is unknown. Associated triggers include vasoconstrictive medications, illicit drugs, sexual orgasm, and recent pregnancy, among others. Most patients present with…

Inflammatory and Infectious Vasculopathies

Key Points Coronavirus disease 2019 (COVID-19), the pandemic illness caused by a novel coronavirus, has been associated with stroke, possibly related to thrombophilia and severe inflammation. Primary angiitis of the central nervous system (PACNS) is an inflammatory arterial disease restricted to the cerebral circulation that can afflict patients of any age, and often responds poorly to steroids alone. Patients with PACNS commonly present with a combination…

Arterial Dissection, Fibromuscular Dysplasia, and Carotid Webs

Key Points Cervicocerebral arterial dissection constitutes 2% of strokes occurring most frequently in young and middle-aged populations. Medical interventions depending on the timing and severity of initial presentation and site of dissection may include thrombolytics, antiplatelet agents, or anticoagulation. Clinical prognosis of extracranial dissection is associated with excellent outcome in 70%–85% of cases with mortality less than 5%. Fibromuscular dysplasia (FMD) is an uncommon nonatheromatous, noninflammatory…

Stroke Related to Surgery and Other Procedures

Key Points Periprocedural stroke accounts for at least 5% of the approximately 800,000 strokes that occur each year in the United States, although these events are likely underreported. Stroke can occur after a wide range of surgeries, and when it occurs, it is associated with substantial morbidity, mortality, and cost. Surgeries with high risk of periprocedural stroke include carotid revascularization, intracranial vascular procedures, cardiac valve surgery,…

Atherosclerotic Disease of the Proximal Aorta

Key Points Large or complex atherosclerotic plaques in the proximal segment of the aorta are a potential cause of ischemic stroke in individuals older than age 60 years. The magnitude of the risk depends on the morphologic characteristics of the plaque (thickness, ulceration, presence of mobile components). Transesophageal echocardiography (TEE) is the most widely used and sensitive diagnostic technique for their detection, but magnetic resonance imaging…

Cardiac Diseases

Key Points Stroke due to cardiac embolism is commonly of sudden onset, is more prone to hemorrhagic transformation, and is associated with higher mortality than other stroke subtypes. The etiologic workup for stroke due to a cardiogenic source is crucial in determining appropriate secondary prevention. Anticoagulation is the mainstay of therapy for patients with cardioembolic stroke secondary to atrial fibrillation, absent any contraindications. Some cardioembolic sources,…

Stroke and Other Vascular Syndromes of the Spinal Cord

Key Points Spinal cord stroke presents with painless paraparesis with urinary retention. The majority of spinal cord stroke can be attributed to diseases of the aorta. Spinal cord infarction occurs most frequently as a complication of aortic dissection and surgery for diseases in the aorta. Prevention of spinal cord infarction may occur with lumbar drainage at the time of aortic surgery. Immobility and urinary retention are…

Arteriovenous Malformations and Other Vascular Anomalies

Key Points Intracerebral vascular malformations constitute an important cause of intracranial hemorrhage in younger patients. The spectrum of vascular malformations ranges from sporadic congenital lesions such as brain arteriovenous malformations (AVMs) to genetically determined familial disorders that may progress over time. Arterial aneurysms, moyamoya disease, and arteriovenous dural fistulas are considered acquired lesions. Developmental venous anomalies represent a variant of physiologic venous drainage and pose minimal,…

Aneurysmal Subarachnoid Hemorrhage

Key Points Subarachnoid hemorrhage (SAH) is a neurologic emergency. Severe and sudden headache is the most common presentation, but patients can experience loss of consciousness or seizures at onset. Most patients with nontraumatic SAH will harbor a ruptured cerebral aneurysm. Patients with SAH need to be evaluated immediately and admitted to an intensive care unit environment. The most frequent and dreaded neurologic complications associated with SAH…

Intracerebral Hemorrhage

Key Points Spontaneous intracerebral hemorrhage (ICH) is the most serious form of acute stroke, which causes enormous loss of life and suffering on a global scale but particularly so in older and socioeconomically disadvantaged populations where the prevalence of hypertension, unhealthy lifestyles, and antithrombotic use are high. Although there are few clearly proven treatments for ICH, an urgent approach is required for diagnosis and management with…

Lacunar Syndromes, Lacunar Infarcts, and Cerebral Small-Vessel Disease

Key Points Lacunar infarcts are small (<15 mm diameter) subcortical infarcts that result from occlusion of a single penetrating artery. Lacunar infarcts account for approximately one-quarter of all ischemic strokes. Lacunar infarcts are associated with clinical lacunar syndromes, but the specificity of the syndromes is only moderate: lacunar syndromes may be caused by other mechanisms than small-artery disease in one-third or more of all cases. Lacunar…

Vertebrobasilar Disease

Key Points Large artery atherothrombosis, cardiac embolism, and small artery disease are the important mechanisms of infarction in vertebrobasilar artery territory. In lateral medullary infarction, clinical manifestations vary according to rostro-caudal and ventrodorsal topography. Rostral-ventral infarcts are associated with severe dysphagia, dysarthria, and contralateral trigeminal sensory involvement, whereas caudal-lateral lesions are characterized by severe gait ataxia, absent dysphagia, and sensory symptoms worse in the lower extremities.…

Posterior Cerebral Artery Disease

Key Points Large artery atherothrombosis, cardiac embolism, and small-artery disease are important stroke mechanisms of posterior cerebral artery territory infarction. The clinical features of thalamic infarction vary according to vascular topography: the inferolateral, tuberothalamic, paramedian, and posterior choroidal arteries. Inferolateral territory infarction is the most common type of thalamic infarction, and the main clinical features are hemisensory deficits. The main clinical features of tuberothalamic and paramedian…

Middle Cerebral Artery Disease

Key Points The middle cerebral artery (MCA) is the largest cerebral artery, and it is the most commonly affected intracranial vessel in cerebrovascular accident. It supplies most of the outer convex brain surface, nearly all the basal ganglia, and the anterior and posterior limbs of internal capsule. Infarcts that occur within the distribution of MCA territory lead to diverse neurologic sequelae. In general, the clinical features…

Anterior Cerebral Artery Disease

Key Points Infarction of the territory of the anterior cerebral artery (ACA) can be the result of carotid artery atherosclerosis and embolism, cardioembolism, local ACA atherosclerosis, or ACA dissection. Considerable variation describes the anatomy of the ACA and the brain regions it supplies. Neurologic impairments following infarction in the ACA territory include weakness, sensory loss, apraxia and callosal disconnection signs, akinetic mutism and motor neglect, language…

Carotid Artery Disease

Key Points Carotid disease is a common manifestation of focal atherosclerosis most often located at the origin of the internal carotid artery, and accounts for 10%–20% of ischemic strokes. Intracranial carotid disease is more common than extracranial disease in Asian, black, and Hispanic populations. The most common mechanism of stroke involves rupture of the atherosclerotic plaque with subsequent embolism to the brain hemisphere or eye. Carotid…