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  1. Home
  2. Books
  3. Uncommon Causes of Stroke
  • Cited by 19
Publisher:
Cambridge University Press
Online publication date:
January 2010
Print publication year:
2008
Online ISBN:
9780511544897
DOI:
https://doi.org/10.1017/CBO9780511544897
Subjects:
Neurology and Clinical Neuroscience, Medicine
Information

Book description

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.

Awards

Highly Commended at the BMA Book Awards 2009

Reviews

Review from the first edition:' … stands alone as a readable text … There is no doubt in my mind that this is a book worth having. I would recommend buying a personal copy and predict that it will become a standard text to be found in all departmental libraries.'

Source: Neuroradiology

'This is an excellent resource and a keepsake in the hospital or professional library of any neurologist, neuroradiologist, neurosurgeon, vascular surgeon, angiologist, as well as stroke physician, pediatrician, and internist.'

Nano Khilnani Source: Biz India

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Contents

Page 2 of 3

Contents
  • 23 - PROGERIA
    pp 145-148
  • View abstract

    Summary

    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.
  • 25 - STURGE-WEBER SYNDROME
    pp 155-162
  • View abstract

    Summary

    Hereditary hemorrhagic telangiectasia (HHT) also known as Osler-Weber-Rendu syndrome is one of the most common autosomal dominant disorders. Three different phenotypes of vascular malformations have been differentiated in the central nervous system (CNS), including large fistulae characterized by a direct arteriovenous shunt without nidus but with an ectatic draining vein, small arteriovenous malformations (AVMs) with a nidal size between 1 and 3 cm, and micro-AVMs with a nidus smaller than 1 cm. Cerebral ischemic manifestations are severe complications of the paradoxical embolism in patients with HHT. Apart from paradoxical embolism, two other mechanisms could favor cerebral ischemic manifestations in HHT patients, including blood hyperviscosity related to polycythemia and air embolism from the lung to the brain. Prevalence of cerebrovascular malformations in HHT is estimated at 5% using only computed tomography (CT), but it increases up to approximately 20% when magnetic resonance imaging (MRI) is performed.
  • 26 - VON HIPPEL-LINDAU DISEASE
    pp 163-170
  • View abstract

    Summary

    Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited small artery disease of mid-adulthood caused by mutations of the NOCTH3 gene on chromosome 19. Ischemic manifestations are the most frequent clinical events in CADASIL: 60-85% of patients have had transient ischemic attacks (TIAs) or complete strokes. Magnetic resonance imaging (MRI) shows on T2-weighted images widespread areas of increased signal in the white matter associated with focal hyperintensities in the basal ganglia, thalamus, and brainstem. Diagnostic testing with immunostaining using anti-NOCTH3 antibodies is an alternative method that seems easier than electronmicroscopy and particularly useful before initiating a complete screening of the gene in difficult cases. As CADASIL is a vascular disorder responsible for cerebral ischemic events, different authors prescribe aspirin for secondary prevention, but its benefit in the disease has not been shown. Treatment of migraine should be restricted to analgesic agents and nonsteroidal anti-inflammatory drugs.
  • 27 - ANEURYSMS
    pp 171-180
  • View abstract

    Summary

    Fabry's disease (FD) or angiokeratoma corporis diffusum, is a rare X-linked inherited disorder of glycosphingolipid metabolism. This chapter analyzes the clinical, radiologic, and pathologic features of hemizygote and heterozygote FD patients and cerebrovascular involvement based on a comprehensive review of literature. Neuropathologic autopsy findings are consistent with prior events of cerebral ischemia and, rarely, hemorrhage. In FD, in addition to cerebral ischemia, dolichoectatic intracranial arteries may also cause neurovascular compression syndromes. Triventricular hydrocephalus related to dolichoectatic basilar artery has been reported in hemizygotic and heterozygotic patients. Magnetic resonance imaging (MRI) studies show progression of white-matter lesion load despite enzyme replacement therapy (ERT), although these patients did not show clinical neurologic progression. Administration of antiplatelet agents may help to prevent the atherosclerotic and thromboembolic effects of damage to the vascular endothelium, but experience with this approach is limited and the use of oral anticoagulant agents should also be considered.
  • 28 - ARTERIOVENOUS MALFORMATIONS OF THE BRAIN
    pp 181-188
  • View abstract

    Summary

    Marfan's syndrome is a connective tissue disorder responsible for an extensive and generalized malformation of organs and systems. An estimate of the risk of developing a cerebrovascular event in Marfan's syndrome is entirely elusive, both in general and for a particular patient. Severity of the vascular malformations differs from patient to patient and, in the worst cases, the chance of a disastrous event is largely related to other than neurological causes. Valvular dysfunction and disturbances of cardiac rhythm can produce embolic strokes basically no different from any other embolic stroke. Intracerebral aneurysms and aneurysmal rupture have for a long time been considered frequent complications of Marfan's syndrome. However, there are currently no prophylactic or curative medical treatments for the crucial Marfan's anomalies. Additional progress in understanding genetics and biochemical defects and in the elucidation of the ultimate mechanisms related to malformations in Marfan's syndrome are expected in the near future.
  • 29 - CEREBRAL CAVERNOUS MALFORMATIONS AND DEVELOPMENTAL VENOUS ANOMALIES
    pp 189-220
  • View abstract

    Summary

    Pseudoxanthoma elasticum (PXE) is an inherited connective tissue disorder, characterized predominantly by skin, eye, cardiac, and vascular abnormalities. The genetic defect has been mapped to the ABCC6 gene on chromosome 16. The most characteristic and diagnostic feature of PXE is the angioid streaks found in the retina. The streaks are red-brown or gray, are usually wider than veins, and radiate from the optic disc. Abdominal angina and ischemic bowel disease occasionally develop. Hypertension is also very common in patients with PXE and often contributes to the cardiac and cerebrovascular pathology leading to strokes. Premature occlusive cervicocranial disease and aneurysmal subarachnoid hemorrhage are the two cerebrovascular problems directly attributable to PXE. It is difficult in patients with Binswanger-like abnormalities, PXE, and hypertension to know how much of the abnormalities, if any, relate directly to PXE and how much are attributable to the hypertension.
  • 30 - CEREBROVASCULAR MANIFESTATIONS OF NEUROFIBROMATOSIS
    pp 221-224
  • View abstract

    Summary

    This chapter contains detailed, up-to-date information about the nature, diagnosis, and treatment of Ehlers-Danlos syndromes (EDS) that cause strokes. The EDS are a group of connective tissue diseases classically characterized by fragile or hyperelastic skin, hyperextensible joints, vascular lesions, and easy bruising and excessive scarring after an injury. Numerous mutations of the COL3A1 gene on chromosome 2, including point mutations, exon skipping mutations, and multi-exon deletions, have been attributed to this disease. EDS patients develop arterial dissections. The cerebrovascular complications include intracranial aneurysms, arterial rupture, carotid-cavernous fistulae, and arterial dissections. The intracranial internal carotid artery is the most common site for an aneurysm, and rupture of the internal carotid artery within the cavernous sinus can create a direct carotid-cavernous fistula. The fragile arteries make angiography and surgery difficult, but some patients have had successful surgery or endovascular treatment.
  • 31 - MENKES DISEASE (KINKY HAIR DISEASE)
    pp 225-230
  • View abstract

    Summary

    Progeria is a rare condition characterized by premature aging beginning in very early life and invariably ending in premature death. Progeria is caused by a mutation of the LMNA gene on chromosome1q. Based on clinical descriptions and, more recently, genetic analyses, several progeroid syndromes have been defined in addition to progeria itself. The manifestations of these conditions vary, but each has some clinical features that resemble physiologic aging and a variable risk of stroke. Most children with progeria develop premature severe vascular disease. Coronary artery disease and myocardial infarction are common, and heart disease is the leading cause of death. The Wiedemann-Rautenstrauch syndrome, sometimes called neonatal progeria, typically manifests from birth and features delayed development, poor growth, alopecia, and lipoatrophy. Given the severity of large artery pathology in patients with progeroid syndromes, it is probably reasonable to use antiplatelet agents.
  • 32 - WYBURN-MASON SYNDROME
    pp 231-234
  • View abstract

    Summary

    Stroke-like episodes have most frequently been reported in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), a multisystemic syndrome associated with mutations of mitochondrial DNA (mtDNA). Blood lactate levels are increased because of a dysfunction in the respiratory chain, with resulting inhibition of the citric acid cycle and accumulation of pyruvate and lactate. Stroke-like episodes have been reported to be associated with other mtDNA mutations. These mutations are point mutations lying within tRNA-encoding genes or within proteinencoding genes, or are deletions encompassing several genes, showing that all these different genetic anomalies can cause a similar dysfunction of the mitochondrion resulting in stroke. Brain imaging can show calcifications of the basal ganglia and focal lesions in the occipital and parietal lobes that are not usually restricted to a vascular territory. NMR spectroscopy detects increased lactate levels. Published therapies include coenzyme Q10, nicotinamide and coadministration of cytochrome c, vitamin B1, and B2.
  • 33 - EALES RETINOPATHY
    pp 235-236
  • View abstract

    Summary

    Sturge-Weber syndrome is characterized by a facial cutaneous nevus (port-wine stain) and a leptomeningeal angioma, often found ipsilateral to the facial lesion. Epileptic seizures, mental retardation, and focal neurological deficits are the primary neurologic abnormalities of Sturge-Weber syndrome. In a series of 52 adults with Sturge-Weber syndrome, 65% had neurologic deficits including stroke, hemiparesis, spasticity, and/or weakness. Neuroimaging, electroencephalography, and functional testing with Positron emission tomography (PET) and Single-photon emission computed tomography (SPECT) may also help to define the extent of the intracranial lesion for possible epilepsy surgery. Although gyral calcification is a classic feature of Sturge-Weber syndrome, this "tram track" appearance is not always present. Bilateral calcification is common. Calcification often becomes more apparent as the patient becomes older but is sometimes already present at birth. Daily aspirin has been tried in an effort to prevent recurrent vascular thrombosis that may cause neurologic deterioration.
  • 34 - ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY
    pp 237-246
  • View abstract

    Summary

    Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder with a 95% penetrance at age 60 and incomplete expression that is characterized by the development of various benign and malignant tumors and cysts. Hemangioblastoma (HB), the most characteristic central nervous system (CNS) lesions, are highly vascular tumors comprising approximately 3% of all tumors of the CNS. HBs are diagnosed by magnetic resonance imaging (MRI) of the brain and the spinal cord. Presentation of HBs with a stroke syndrome is rare despite their highly vascular nature, and the risk of hemorrhage associated with them is not yet known. Screening for VHL disease following the diagnosis of a cerebral HB is indispensable given the high risk of associated lesions with hereditary forms. The best treatment for this tumor is surgical resection. If the tumor is difficult to remove from its primary site, it can sometimes be treated with radiosurgery.
  • 35 - MICROANGIOPATHY OF THE RETINA, INNER EAR, AND BRAIN: SUSAC’S SYNDROME
    pp 247-254
  • View abstract

    Summary

    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.
  • 36 - HEREDITARY ENDOTHELIOPATHY WITH RETINOPATHY, NEPHROPATHY, AND STROKE (HERNS)
    pp 255-258
  • View abstract

    Summary

    Arteriovenous malformations (AVMs) are complex vascular lesions that typically present with hemorrhage or seizures. Computed tomography (CT) scan, magnetic resonance imaging (MRI), and conventional cerebral angiography are the standard essential diagnostic tools utilized in planning the treatment of an AVM. Brain AVMs are complex cerebrovascular lesions capable of protean symptomatology. They are an infrequent, but serious cause of stroke that often occurs in young people. Most commonly, they result in hemorrhagic stroke by bleeding into the parenchyma of the brain. Much less commonly, they result in subarachnoid hemorrhage. Ischemic stroke is distinctly uncommon with cerebral AVMs but occurs occasionally due to retrograde thrombosis of feeding arteries frequently associated with complete or partial thrombosis of the AVM. The three treatment modalities available to patients with cerebral AVMs, embolization, radiosurgery, and surgical excision, should be carefully considered in each patient and should not be thought of as being interchangeable.
  • 37 - COGAN’S SYNDROME
    pp 259-262
  • View abstract

    Summary

    There are four types of cerebral vascular malformations: arteriovenous malformations (AVMs), cavernous malformations (CMs), capillary telangiectasies, and venous malformations. This chapter presents a review of the pertinent literature on CMs regarding epidemiology, genetics, pathology, clinical findings, and therapeutic management with special emphasis placed upon the natural bleeding risk of these malformations. The most sensitive imaging study to detect CMs is magnetic resonance imaging (MRI). Predictive factors for intracranial hemorrhage in patients harboring CMs is a critical issue because the optimal therapeutic management of such lesions is tailored according to the bleeding risk. The main goal of radiosurgical treatment should be a significant reduction in bleeding risk, especially after a latency period of 2 years. By genetic linkage analyses, three cerebral CM loci have been assigned to chromosome 7p, 7q, and 3q. They account for all familial forms of CM, thus constituting a formidable Mendelian model of stroke.
  • 38 - ANTI-PHOSPHOLIPID ANTIBODY SYNDROME
    pp 263-274
  • View abstract

    Summary

    Neurofibromatosis (NF) is an autosomal dominant disorder encompassing several distinct genetic defects with overlapping clinical features. This chapter focuses on the main genetic, clinical, pathological, and radiological features of neurofibromatosis type 1 (NF1). The cerebrovascular manifestations of NF1 include cerebral ischemia, intracranial hematoma, and subarachnoidal hemorrhage. Recurrent strokes can occur in the same or in different territories. Every large or medium-sized artery can be affected, and some patients have multiple stenoses of intracranial vessels combined with stenoses or occlusion of extracranial vessel. Hemispheric territorial infarction is a common stroke manifestation. Ocular involvement may present with retinal ischemia or global ocular ischemia. Angiography, time-of-flight magnetic resonance angiography (TOF-MRA) and Color duplex and spectral Doppler ultrasonography are useful diagnostic modalities for revealing the vascular complications of NF1. Neurosurgical revascularization in patients with moyamoya syndrome has been shown to reduce the risk of first-ever and recurrent stroke and transient ischemic attack (TIA).
  • 39 - DISSEMINATED INTRAVASCULAR DISEASE
    pp 275-282
  • View abstract

    Summary

    The characteristic feature of Menkes disease (MD), as expressed in the human infant, is maldistribution of body copper. It is caused by mutation in ATP7A, a gene mapped to the long arm of the X-chromosome (Xq12-q13). This chapter focuses on pathology, clinical manifestations, diagnosis and treatment options for Menkes disease (MD). The clinical history and changes within the brain result from vascular lesions, copper deficiency, or a combination of the two. On angiographic or magnetic resonance (MR) angiographic studies reveal, a striking and progressive intracranial and extracranial vascular tortuosity is apparent. Similar changes are seen in the systemic vasculature. Aneurysms are not unusual. Neuroimaging discloses cerebral atrophy and bilateral ischemic lesions in deep gray matter, or in the cortical areas, the consequence of brain infarcts. Copper supplementation, using daily injections of copperhistidine, appears to be the most promising treatment.
  • 40 - BLEEDING DISORDERS AND THROMBOPHILIA
    pp 283-300
  • View abstract

    Summary

    Wyburn-Mason syndrome (WMS), also known as the Bonnet-Dechaume-Blancsyndrome, is a rare non hereditary phakomatosis characterized by congenital retinal, orbital, and brainstem (usually midbrain) arteriovenous malformations (AVMs), and, less frequently, facial AVMs. With the increased availability of noninvasive brain imaging, intracranial AVMs are detected. Recognition of the association between the retinal and intracranial lesions is important because it allows early identification of the associated intracranial and facial AVMs. This chapter discusses the historical features, pathophysiology, and treatment options for WMS. Retinal AVMs usually do not grow or bleed, and are usually not responsible for significant visual loss. Due to their size and location, WMS AVMs are usually not amenable to surgical removal or radiosurgery. Embolization carries an increased risk because the lesions share a blood supply with vital brainstem structures. Therefore, patients are usually left untreated until the AVMs bleed, at which time heroic measures may be necessary.
  • 41 - THROMBOTIC THROMBOCYTOPENIC PURPURA
    pp 301-308
  • View abstract

    Summary

    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.
  • 43 - MICROSCOPIC POLYANGIITIS AND POLYARTERITIS NODOSA
    pp 311-330
  • View abstract

    Summary

    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.
  • 44 - CHURG-STRAUSS SYNDROME
    pp 331-334
  • View abstract

    Summary

    This chapter explores the relationship of stroke with Susac's syndrome. In Susac's syndrome, diagnostic signs involve the retina. Fundoscopic examination shows arteriolar occlusions with narrowing of arterioles, as well as signs of other ischemic changes in the affected vascular area, such as edema and increased vascular permeability. Fluorescein angiography is often helpful in showing the vascular occlusions and leaking into the retina. Magnetic resonace imaging (MRI) is the neuroimaging study of choice and fluid-attenuated inversion recovery (FLAIR) a sensitive sequence for detecting lesions of Susac's syndrome as well as to show their heterogeneity. Small-vessel diseases are responsible for a large amount of ischemic and hemorrhagic strokes as well as encephalopathies including Susac's syndrome. Misdiagnoses include multiple sclerosis, but differential diagnosis must be done with all causes of multifocal neurologic symptoms with hearing and/or visual loss. Calcium channel blockers (nimodipine), anticoagulants, and aspirin may be useful in treatment.
  • 45 - SYSTEMIC LUPUS ERYTHEMATOSUS
    pp 335-342
  • View abstract

    Summary

    Hereditary endotheliopathy with retinopathy, nephropathy, and stroke (HERNS) typically begins with progressive visual loss in the third or fourth decade of life followed by focal neurological deficits within 4-10 years. The clinical features of HERNS include strokes, retinopathy, nephropathy, migraine, mood disorders, and dementia. The underlying mechanism of HERNS appears to be a generalized vasculopathy with disruption of the integrity of capillaries and arterioles. Fluorescein angiograms clearly show retinal vasculopathic changes. That the intracerebral lesions show contrast enhancement on magnetic resonance imaging (MRI) indicates break down in the blood-brain barrier. The surrounding edema in a vasogenic pattern also suggests increased capillary permeability. At the present time there is no known treatment that is effective in patients with HERNS. However, corticosteroids have been useful to decrease cerebral edema and may even be life-saving in patients with large edematous lesions.
  • 46 - RHEUMATOID ARTHRITIS AND CEREBROVASCULAR DISEASE
    pp 343-346
  • View abstract

    Summary

    Cogan's syndrome is characterized by nonsyphilitic interstitial keratitis, vestibulo-auditory Menière-like symptoms, and, occasionally, systemic manifestations of vasculitis. Although neurologic manifestations are rare, several patients with stroke in the setting of Cogan's syndrome have been reported. The most common and classic ocular manifestation of Cogan's syndrome is bilateral interstitial keratitis. The diagnosis is classically suggested by the association of interstitial keratitis with acute-onset sensorineuronal hearing loss in a patient who has a negative laboratory evaluation for syphilis. Computed tomography (CT) scans may occasionally show intralabyrinthine calcifications, whereas magnetic resonance imaging (MRIs) often show soft tissue obliteration of the membranous labyrinth and may also show multiple lesions of the white matter consistent with cerebral vasculitis. The treatment of Cogan's syndrome varies based on the severity of the clinical manifestations. Due to the presumed autoimmune mechanism with vasculitis, most treatments have included steroids and immunosuppressants.
  • 47 - HYPERVISCOSITY AND STROKE
    pp 347-356
  • View abstract

    Summary

    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.
  • 49 - STROKE AND SUBSTANCE ABUSE
    pp 365-370
  • View abstract

    Summary

    Disseminated intravascular coagulation (DIC) is a disorder of clotting caused by a cytokine-induced systemic inflammatory response that results in consumption of platelets, coagulation factors, and tissue factor plasma inhibitors (TFPIs). There is a wide differential diagnosis that includes thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome, systemic lupus erythematosus (SLE), catastrophic antiphospholipid syndrome (APS), and Evans syndrome. A peripheral blood smear in DIC often shows schistocytes, fragmented red blood cells (RBCs) that are formed by fibrin RBC adhesion. DIC is a major cause of stroke in medical intensive care units and is a frequent complication of a terminally ill patient. Replacement therapy with platelets and coagulation factors is given to those patients with severe bleeding. Antithrombin III is the most important of the thrombin inhibitors. It has a beneficial effect in improving coagulation factors and organ function and decreasing mortality in the majority of DIC clinical trials.

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