Physical Address

304 North Cardinal St.
Dorchester Center, MA 02124

Moyamoya Disease and Syndrome

Introduction Moyamoya disease (MMD) is a chronic, occlusive cerebrovascular disease with an unknown etiology. It is characterized by steno-occlusive changes at the terminal portion of the internal carotid artery (ICA), and an abnormal vascular network at the base of the brain . When there are causative diseases or associated conditions, the terms “moyamoya syndrome” and “angiographic moyamoya” are often used . Epidemiology MMD is relatively common…

Stroke in Fabry Disease

Fabry disease (FD), also known as angiokeratoma corporis diffusum , is an X-linked lysosomal storage disorder caused by deficiency of α-galactosidase A (α-gal). This defect causes the accumulation of glycosphingolipids in cells, including endothelial and vascular smooth muscle cells of small arterioles. These vessels become dysfunctional and undergo progressive stenosis resulting in ischemia and end-organ damage. Stroke constitutes one of the most devastating complications of FD.…

Rare Genetic Causes of Stroke

Introduction Cerebrovascular diseases constitute the fourth leading cause of death in the United States annually and the third leading cause of mortality in developed countries . In addition, it is the number one cause of permanent disability globally and the second most common cause of dementia . Many risk factors for cerebrovascular diseases have been established including nonmodifiable factors such as age, gender, and race, as…

Ischemic Stroke in the Young

Introduction Age constitutes one of the most important nonmodifiable risk factors for cerebral infarction. About 15% of all the ischemic strokes occur in young adults. Since stroke predominately affects the elderly, most of the data gathered in pivotal clinical trials and population-based studies were obtained using cohorts of older patients with coexisting cardiac disease and atherosclerosis. In comparison to older individuals, however, patients aged 15–50 years…

Stroke in Children

Introduction There are various types of stroke in children, including arterial ischemic strokes, venous infarctions due to venous sinus thrombosis, intracerebral hemorrhages, and subarachnoid hemorrhages. Stroke is a relatively rare occurrence in children, but can lead to significant morbidity and mortality, and is one of the top 10 causes of death in children aged 1–18 years . It is important to be aware of the fact…

Vascular Cognitive and Behavioral Disorders

Introduction Ischemic infarcts in the brain can manifest with a variety of cognitive and behavioral disorders. For many years, use of autopsy and clinical–pathological correlation in stroke patients formed the basis for the anatomic localization of various cognitive functions. In addition to focal infarcts, diffuse cerebrovascular disease can also be associated with subcortical white matter injury and secondary vascular dementia. A full description of vascular dementia…

Stroke Secondary to Trauma

Introduction Stroke is common following trauma. Indeed, direct cerebrovascular injury during a traumatic event can often lead to immediate ischemic and hemorrhagic complications. However, recent definitions for hemorrhagic stroke have, by policy, excluded etiologies that are secondary to trauma . This was done to emphasize differences in presentation and outcomes between traumatic and nontraumatic etiologies, with nontraumatic etiologies including arterial hypertension, vascular malformation, underlying mass lesion,…

Spontaneous Dissections of Cervicocephalic Arteries

Etiology and Physiopathology Dissection is a focal arteriopathy caused by a hematoma inside the arterial wall. It occurs most commonly in the extracranial and most mobile segments of the internal carotid and vertebral arteries. The specific vulnerability of this particular vascular segment to trauma is probably related to structural anatomical factors. The extracranial carotid segment is freely movable with a fixation at the entry into the…

Reversible Cerebral Vasoconstriction Syndromes

Introduction The term “ reversible cerebral vasoconstriction syndrome ” (RCVS) refers to a group of conditions characterized by segmental narrowing and dilatation of multiple intracerebral arteries lasting days to weeks, usually accompanied with recurrent thunderclap headaches [ ]. Historically, patients with RCVS have been misinterpreted as having primary angiitis of the central nervous system (PACNS) or subarachnoid hemorrhage from ruptured brain aneurysms due to overlapping features…

Spinal Vascular Malformations

Vascular malformations of the spinal cord are a heterogeneous group of anomalies ranging from simple, dural arteriovenous fistulae to complex arteriovenous malformations that may involve the spinal cord parenchyma as well as adjacent spinal and extraspinal structures. Although spinal vascular malformations are uncommon, they represent a potentially curable cause of progressive myelopathy. As with most uncommon disorders, a high index of suspicion is necessary to make…

Cavernous Malformations

Introduction Cavernous malformations (CMs) are the second most common form of vascular malformation of the central nervous system after developmental venous anomalies (DVAs). The incidence of CMs is 0.34–0.53% in autopsy series and 0.39–0.47% in MRI series . The overall male to female ratio is 1:1 and the mean age at presentation is 30.6 years . CMs can occur sporadically or in a familial form. In…

Carotid Cavernous and Other Dural Arteriovenous Fistulas

Introduction Intracranial dural arteriovenous fistulas (dAVFs) are acquired vascular lesions that usually involve the intracranial dural sinuses and comprise less than 10% of all intracranial vascular lesions . They are commonly divided into carotid cavernous fistulas (CCFs) and other dAVFs. Usually branches of the external carotid artery (ECA), internal carotid artery (ICA), or vertebral artery or a combination thereof form a direct connection with dural sinus…

Vein of Galen Arteriovenous Malformations

Vein of Galen arteriovenous malformations (VOGMs) are a rare congenital vascular anomaly in which an arteriovenous shunt is established to the median prosencephalic vein of Markowski (MPV), a fetal vein typically present between weeks 7 and 12 of gestation . Although true incidence of the condition is difficult to ascertain, VOGMs represent the earliest-appearing pediatric cerebrovascular malformation . Other than a minority of patients, in whom…

Developmental Venous Anomalies

Introduction Developmental venous anomalies (DVAs) are rare, usually asymptomatic congenital cerebrovascular malformations consisting of a radial network of medullary veins draining into a dilated central channel. Originally called venous angiomas, DVAs are functional variants of normal venous anatomy that drain brain parenchyma, which is better described by the DVA nomenclature . DVAs have a 2.5% incidence in postmortem studies and are the most common developmental vascular…

Cerebral Venous Thrombosis

Introduction Cerebral venous thrombosis (CVT) accounts for 0.5–1% of all strokes. CVT is more frequent in children and young adults and affects women three times more than males. Its clinical presentation is diverse and diagnosis is often challenging. CVT may arise in several clinical settings, such as pregnancy or puerperium, malignancy, head or neck infections, inflammatory disorders, and after some diagnostic and therapeutic procedures. CVT is…

Clinical Aspects of Subdural Hemorrhage (SDH)

Acute Subdural Hematoma Epidemiology Acute subdural hematoma (ASDH) is an intracranial space-occupying lesion that often occurs because of the tearing of bridging or cortical surface veins, secondary to a physical head trauma. ASDHs are often accompanied by cortical contusions, parenchymal hematomas, or global shearing injury such as diffuse axonal injury (DAI). Approximately 10–20% of all patients admitted with a traumatic brain injury (TBI) have an ASDH.…

Clinical Aspects of Intraventricular Hemorrhage

Introduction Intraventricular hemorrhage (IVH) is the presence of blood within the ventricular system including the lateral, third and fourth ventricles. Primary IVH refers to bleeding directly into the ventricular system within the brain, from an intraventricular source or a lesion contiguous to the ventricles. Primary IVH is uncommon. Secondary IVH occurs more frequently (70% of IVH patients) and refers to bleeding extending from the parenchyma or…

Clinical Aspects of Intracerebral Hemorrhage

Introduction Stroke is the second most frequent cause of death in the world, with 6.7 million globally documented cases in 2012 alone. This chapter focuses on intracerebral hemorrhage (ICH), the least common type of stroke, accounting for about 13% of all stroke victims, occurring at an annual rate of about 12–15 cases per 100,000 people. Despite having the lowest incidence of any type of stroke, the…

Clinical Aspects of Subarachnoid Hemorrhage

Introduction Aneurysmal rupture leading to subarachnoid hemorrhage (SAH) represents a dire clinical entity with profound neurologic and systemic manifestations. Despite several advances in microsurgical and endovascular technology, mortality rates remain high, and survivors often suffer substantial neurologic morbidity; of all patients with aneurysm rupture, 25% die within the first 24 h and nearly half die within 30 days . As such, proper diagnostic evaluation and early…

Unique Features of Aneurysms by Location

Introduction The rupture of intracranial aneurysms is associated with severe morbidity and mortality. Prehospital mortality estimates range from 10% to 15% . The rate of rerupture if untreated is 40% over 4 weeks with much of that risk early on; rerupture carries an 80% mortality underscoring the importance of early treatment for ruptured aneurysms. In patients who survive the initial hemorrhage long enough to undergo treatment,…