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Prospective studies of mood changes after stroke in large samples of patients may permit the delineation of the acute emotional behavioral changes that are markers for the delayed development of emotional disturbances. Mood disorders can be quantified using specifically designed scales, such as the Center for Epidemiological Studies-Depression (CES-D) or the Hospital Anxiety and Depression Scale (HADS), and may be predictors of the later development of depression. The standardized diagnostic criteria of the DSM-IV for mood disorders are appropriate for stroke, as poststroke depression has a similar symptomatic profile to primary depression. Fear and anxiety are common following stroke. Anxiety is the second most prevalent mood disorder following stroke, being found in 3.5%-24% of patients. Careful monitoring of stroke and measurement of monoamine metabolites and neuroexcitatory amino acids, may give a better understanding of the biological mechanism underlining poststroke emotional disturbances.
Intravascular lymphoma is a rare, extranodal, large B-cell lymphoma in which neoplastic, lymphoid cells proliferate within the lumina of small to medium-sized vessels. Neurological findings develop in about two-thirds of patients and usually present as multifocal cerebrovascular events, subacute encephalopathy, spinal cord or nerve root vascular syndromes, or peripheral and cranial neuropathies. In the brain, intravascular lymphoma manifests primarily as multiple infarcts. The strokes may involve any area of the brain, although supratentorial infarcts are more prevalent than cerebellar or brainstem infarction. Encephalopathy is observed in intravascular lymphoma patients and may be accompanied by focal neurological signs or seizures. The peripheral neuropathy observed in intravascular lymphoma is a predominantly axonal neuropathy. Computed tomography (CT) and cerebral angiography may show characteristics of stroke and vessel occlusion, in many instances, these studies are normal. Systemic chemotherapy with anthracycline-based regimens has been the most commonly pursued treatment in the literature.
Most strokes are attributed to atherosclerosis of neck and intracranial arteries, brain embolism from the heart, and penetrating artery disease; these are discussed in detail in many other books. This compendium fills an important niche by providing authoritative discussions on the other, less common causes of stroke, including various forms of angiitis, coagulation disorders, infective, paraneoplastic and metabolic disorders that may be associated with stroke, and a number of rare syndromes such as Eales disease and Fabry's disease. This new edition contains detailed, up-to-date information about the nature, diagnosis, and treatment of those relatively uncommon types of cerebrovascular disease that cause strokes. It is therefore a unique scientific and clinical resource that provides a useful reference to help physicians diagnose and treat stroke patients who do not fit well into the usual clinical categories. New chapters include stroke in patients with Lyme disease, scleroderma, Cogan's syndrome, Chagas' disease, and HIV.
This chapter explores the complex relationship of stroke with polyarteritis nodosa (PAN) and microscopic polyangiitis (MPA). MPA is a systemic necrotizing vasculitis that clinically and histologically involves capillaries, venules, or arterioles without granulomata, and is associated with necrotizing crescentic glomerulonephritis and hemorrhagic pulmonary capillaritis, which are the main causes of mortality and morbidity. Hemorrhagic strokes occur more frequently than ischemic infarction in MPA. Immunohistochemical studies from muscle and nerve biopsies showed that macrophages and T cells, mostly CD8+, are involved in the pathogenesis of PAN. Neurological symptoms and signs are a major and common feature of PAN, occurring in nearly three-quarters of patients. A close relationship between the use of corticosteroids and stroke exists in PAN. From a therapeutic point of view, antiplatelet drugs, which inhibit platelet thromboxane production, might reduce the risk of corticosteroid-induced, antiplatelet drugs in PAN. The use of aspirin and corticosteroids prospectively prevents stroke recurrence.
Cerebral venous sinus thrombosis (CVST), although a relatively rare cause of stroke, is important to diagnose and treat early because of the significant morbidity and mortality associated with it. CVST tends to affect younger patients, who have fewer traditional risk factors, than do arterial infarcts. CVST can also follow traumatic injury to the venous sinuses or jugular veins after neurosurgical or other procedures. Bilateral basal ganglionic and thalamic infarcts or hemorrhages are pathognomic of deep venous involvement. Magnetic resonance imaging (MRI) and computed tomography (CT) scanning are essential in confirming the diagnosis of CVST. The major pathology in CVST is thrombosis of cerebral veins or dural sinuses leading to impaired drainage, venous hypertension with subsequent edema formation, venous infarction, and hemorrhage. Anticoagulation plays a central role in management of intracranial venous occlusive disease. Anticoagulants are likely to prevent further thrombus growth and propagation, helping to arrest the thrombotic process.
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an ophthalmologic syndrome rather than a specific entity, characterized by multiple cream-colored placoid lesions located in the posterior pole "lying at the level of the pigment epithelium and choroids". The ophthalmoscopic hallmarks of APMPPE consist of creamcolored, flat, and discrete placoid, without clear-cut marginal lesions at the level of the retinal pigment epithelium, masking the fundus view of the underlying choroids, which typically involve the macula but are never seen anterior to the equator. The fact that cardiovascular diseases (CVDs) occur in patients with APMPPE strongly supports the thesis that it represents a particular "uveo-cerebral vasculitic syndrome". Various etiologies have been found (infectious/postinfectious; vaccinations; inflammations; autoimmune diseases; vasculitis; paraneoplastic syndrome). The neurological complications of APMPPE are headache, aseptic meningitis, encephalitis, multiple sclerosis-like disease, and pseudo tumor cerebri. CVDs associated with APMPPE consist of ischemic cortical strokes and deep infarcts with striatocapsular infarctions.
The term cervico-cephalic arterial dissection (CAD) encompasses a group of arteriopathies not necessarily with an identical pathogenesis but that have in common an intramural hemorrhage. This most frequently affects the extracranial arterial segments, predominantly the internal carotid artery (ICA), the vertebral artery (VA), or multiple arteries in typical locations. Spontaneous cervico-cephalic arterial dissections (sCAD) have typical predilection sites in the different arteries affected, which in part may be explained by mechanical influences: high cervical segment of extracranial ICA or V2 and V3 segments of VA. In the case of a severe head and/or neck trauma, the pathogenesis is straightforward. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) have replaced angiography as the gold standard, at least in the extracranial segments of ICA and VA. The risk of having a stroke or transient ischemic attack (TIA) after having suffered a CAD in general is very low.
This chapter deals with isolated angiitis of the central nervous system (CNS), and begins with an overview of the pathology and pathogenesis of the condition. The nonspecific pathological pattern of isolated CNS angiitis is characterized by infiltrations of the vascular walls with mononuclear cells including lymphocytes, macrophages, and histiocytes. The pathogenesis of isolated CNS angiitis is unknown and progress is slow because of the rarity of tissue samples acquired from carefully documented cases. Brain imaging, angiography, and brain biopsy are the diagnostic options investigated in the chapter. In patients with a unique focal presentation such as stroke, and with isolated CNS angiitis suspected on the basis of angiography alone, a course of several-weeks of high-dose corticosteroids associated with a calcium channel blocker and no immunosuppressor can be proposed. The diagnosis of reversible cerebral angiopathy should be carefully considered in these patients.
Eales retinopathy associates peripheral retinal changes often described as "vasculitis", peripheral capillary nonperfusion (retinal ischemia), and retinal or optic nerve neovascularization (secondary to chronic retinal ischemia) resulting in vitreous hemorrhage and retinal detachment. Some of the syndromes are associated with lesions of the central nervous system, explaining why Eales retinopathy is considered an uncommon cause of stroke. It should be interpreted with caution because other entities may be the cause of the retinopathy and the central nervous system lesions. Various neurologic findings have been reported, including meningitis, encephalitis, cerebral vasculitis, brain infarctions and hemorrhages, cerebral venous thrombosis, and white matter diseases. The treatment of the retinopathy is limited to stimulating regression of neovascularization by applying laser photocoagulation to the nonperfused retina. Vitrectomy is indicated for nonclearing vitreous hemorrhage, extensive retinal neovascularization, epiretinal membrane, and traction retinal detachment.
Pulmonary arteriovenous malformations (PAVMs) are rare occurrences among the population. These malformations can be acquired in various clinical settings or be congenital. Single PAVMs <2 cm in diameter may be asymptomatic. The severity of symptoms is believed to be proportionally related to the size (diameter) of the PAVMs. The specific anatomy of the vascular malformation must then be analyzed either with helical computed tomography (CT) or magnetic resonance angiography (MRA). Digital subtraction angiography is eventually performed. The aims of treatment are to prevent neurological complications and pulmonary hemorrhage and to improve hypoxemia. Lobectomy or pneumonectomy is performed only in rare situations such as in patients in whom the feeding artery is so short that there is a risk of coil migration. Embolization therapy is applied for PAVMs, and is carried out by transcatheter embolotherapy, which consists in obliterating the feeding artery.
The anti-phospholipid syndrome (APS) was first described in 1983. Anti-phospholipid antibodies form a heterogeneous family that can be detected using a number of immunoreactivity assays. Brain ischemic events in patients with anti-phospholipid antibodies can occur in any vascular territory. Anti-phospholipid antibodies are well established as risk factors in a first ischemic stroke, but their role in recurrent stroke is less clear. Cerebral angiography typically demonstrates intracranial branch or trunk occlusion or is normal in about one-third of patients so studied. Echocardiography (primarily two-dimensional, transthoracic) is abnormal in one-third of patients, typically demonstrating nonspecific left-sided valvular (predominantly mitral) lesions, characterized by valve thickening. Recurrent stroke in patients with livedo reticularis (Sneddon's syndrome) has been associated with anti-phospholipid antibodies. Treatment such as platelet antiaggregant and anticoagulant therapy for secondary stroke prevention have both been used in anti-phospholipid antibody syndrome (AAS) and in cerebrovascular disease associated with antiphospholipid antibody immunoreactivity.
Cerebral vasoconstriction has been associated with several diverse conditions, including pregnancy, vasoconstrictive drugs, and headache disorders. While there may be differences in the etiopathogenesis of cerebral vasoconstriction with each associated condition, the clinical, laboratory, imaging, and angiographic features of these patients are remarkably similar, justifying the use of the inclusive term reversible cerebral vasoconstriction syndromes (RCVS). The RCVS affects women more often than men. The most frequent head CT or brain MRI abnormality is bihemispheric, symmetric infarcts in arterial borderzone regions. Serum calcium, and in some instances parathyroid hormone, levels should be drawn because hypercalcemia is a known cause of cerebral vasoconstriction and can cause strokes. The differential diagnosis primarily includes disorders that present with acute, severe thunderclap headache, which can be excluded with appropriate brain and vascular imaging. Calcium-channel blockers such as verapamil, nimodipine, and nicardipine show more effects on cervico-cranial arteries.
Rupture of an aneurysm is associated with a very high degree of morbidity and mortality. Stroke-like apoplectic clinical syndromes occur with aneurysmal intra cerebral hemorrhage and correspond to the affected area. Lateralized focal neurologic deficits are most common with intra parenchymal hemorrhages due to middle cerebral aneurysms. Intracranial aneurysms are not a rare cause of both hemorrhagic and ischemic stroke. Although the initial and most serious manifestation of an intracranial aneurysm, subarachnoid hemorrhage (SAH), does not typically result in focal neurologic deficits, several complications of ruptured or unruptured aneurysms can lead to focal neurologic deficits, which may develop suddenly in a stroke-like fashion. This is most often seen as a result of intracerebral hemorrhage from the initial rupture of the aneurysm. Cerebral vasospasm after SAH is another common cause of stroke-like, focal deficits. Thromboembolism from the dislodgement of an intra-aneurysmal clot is a less frequent cause of ischemic stroke.