Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
  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

Refine List

Actions for selected content:

Select all | Deselect all
  • View selected items
  • Export citations
  • Download PDF (zip)
  • Save to Kindle
  • Save to Dropbox
  • Save to Google Drive

Save Search

You can save your searches here and later view and run them again in "My saved searches".

Please provide a title, maximum of 40 characters.
Cancel
×

Contents

Page 3 of 3

Contents
  • 50 - CANCER AND PARANEOPLASTIC STROKES
    pp 371-376
  • View abstract

    Summary

    This chapter focuses on the various etiologies for intracerebral hemorrhage (CH), ischemic stroke (IS), transient ischemic attack (TIA), and cerebral venous sinus thrombosis (CVST) that are caused by blood disorders. Bleeding disorders may be inherited or acquired. They include thrombocytopenia and platelet function disorders, coagulation factor deficiencies, excessive anticoagulation and hemorrhagic complications after thrombolysis. Blood disorders associated with myeloproliferative diseases and disseminated intravascular coagulation (DIC) can cause both bleeding and thrombosis. Heparin-induced thrombocytopenia (HIT), the anti-phospholipid antibody syndrome, and thrombotic thrombocytopenic purpura (TTP) are conditions that cause thrombocytopenia but are more frequently responsible for thrombosis than for bleeding. Blood transfusion therapy is recommended as the most important intervention for primary stroke prevention and secondary stroke prevention in children with sickle cell disease. Cytoreduction with hydroxyurea reduces the incidence of thrombosis in essential thrombocythemia, and aspirin reduces the incidence of thrombotic events in polycythemia vera.
  • 51 - KOHLMEIER-DEGOS’ DISEASE (MALIGNANT ATROPHIC PAPULOSIS)
    pp 377-380
  • View abstract

    Summary

    The underlying pathological mechanism of thrombotic thrombocytopenic purpura (TTP) is the presence of microvascular thrombi that partially occlude the vascular lumins with overlying proliferative endothelial cells. Coombs-negative hemolytic anemia and severe thrombocytopenia owing to platelet clumping in the microcirculation are the most outstanding laboratory abnormalities. Classically, TTP has been recognized by the pentad of microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms, fever, and renal involvement, though only 20 to 40 percentage of patients will manifest the classic pentad. TTP remains a life-threatening disease the mortality rate of which may be as high as 90 percentage when untreated. Diagnosis is mainly based on hematological findings and a broad variety of neurological abnormalities, including ischemic or, less often, hemorrhagic stroke. Plasma exchange (PE) is currently the mainstay of treatment; however, rapid advances in the understanding of TTP pathophysiology may offer more specific and effective therapies in the near future.
  • 52 - STROKE IN PATIENTS WHO HAVE INFLAMMATORY BOWEL DISEASE
    pp 381-386
  • View abstract

    Summary

    Henoch-Schönlein purpura (HSP), the most common vasculitis that affects children, is an acute, small-vessel leukocytoclastic process. HSP is a systemic vasculitis involving vascular wall deposits of predominantly immunoglobulin (Ig) A within the small vessels of the gut, skin, joints, and kidneys, and in the mesangium of the renal glomeruli. The most frequent laboratory abnormalities are high erythrocyte sedimentation rates, microscopic hematuria, proteinuria, and elevated levels of IgA. HSP is known to cause neurological complications including seizure, chorea, encephalopathy, focal neurological signs, cortical blindness, as well as cranial and peripheral neuropathies and intracerebral hemorrhage. Ischemic infarction and strokes also occur in HSP. Pulse steroids have been shown to be effective, but sometimes plasmapheresis has been used to arrest disease progressio. Patients with severe nephritis, especially with the nephritic syndrome, have often been treated with corticosteroids and cyclophosphamide, cyclosporine, or azathioprine.
  • 53 - SWEET’S SYNDROME (ACUTE FEBRILE NEUTROPHILIC DERMATOSIS)
    pp 387-390
  • View abstract

    Summary

    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.
  • 55 - EPIDERMAL NEVUS SYNDROME
    pp 401-404
  • View abstract

    Summary

    The Churg-Strauss syndrome (CSS) is a type of small-vessel vasculitis that involves the capillaries, arterioles, and venules. There are different clinical and histopathological criteria for the diagnosis of CSS. The clinical criteria of J. G. Lanham and his group for the diagnosis of CSS is a triad consisting of asthma, peak eosinophilia > 1.5 x 109/L, and systemic vasculitis involving two or more extrapulmonary organs. Glucocorticoids remain the main stay of therapy in CSS. Initial therapy with prednisone is usually pulsed (15 mg/kg over 60 minutes repeated at 24-hour intervals for 1-3 days) followed by prednisone 1 mg/kg per day. Involvement of the central nervous system (CNS) is associated with an increased risk for mortality and requires aggressive treatment with steroids and immunosuppressants. The condition should be considered in any patient who has asthma and develops eosinophilia and peripheral and/or CNS signs.
  • 56 - SNEDDON’S SYNDROME
    pp 405-412
  • View abstract

    Summary

    The risk of recurrence of stroke in patients with systemic lupus erythematosus (SLE) is much higher than in other stroke patients, and the preventative treatment is influenced by the underlying systemic disease. Microinfarcts and microhemorrhages are seen frequently in autopsy specimens of SLE patients. Asymptomatic microinfarcts are common, and are now diagnosed due to the high sensitivity of MRI. Occlusions of large arteries and major strokes also occur in lupus patients. Atherosclerosis may be more frequent in SLE patients than in the general population. The major causes of stroke in lupus are cardiogenic emboli and hypercoagulable (including hypofibrinolytic) states, and the mainstay of stroke prevention is long-term warfarin, with an international normalized ratio (INR) of approximately 3.0. Although stroke is an important problem in lupus patients, leading to significant morbidity in young patients, SLE is relatively uncommon in young patients presenting with strokes or transient ischemic attacks (TIAs).
  • 57 - MITOCHONDRIAL AND METABOLIC CAUSES OF STROKE
    pp 413-422
  • View abstract

    Summary

    Rheumatoid arthritis (RA) is an autoimmune disease that is characterized by an inflammatory, symmetric polyarthritis. The mechanisms by which RA patients are predisposed to stroke are varied. The most common of these causes is atherosclerotic disease, which seems to be accelerated by the underlying RA. The role of systemic inflammation in mediating atherosclerotic disease is becoming clear and may be the underlying link between RA and vascular events. Systemic vasculitis in RA involves the vasa nervorum of the peripheral nervous system. Meningeal and brain biopsy remains the gold standard in confirming a diagnosis of vasculitis in RA patients. Although there is a theoretical risk of mechanical injury to the vertebral artery due to cervical arthritis and atlantoaxial subluxation in RA, this most often results in cervical myelopathy. Stroke due to vertebral dissection has been reported in RA, but the vertebral artery occlusion was sometimes associated with only minimal atlantoaxial subluxation.
  • 58 - BONE DISORDERS AND CEREBROVASCULAR DISEASES
    pp 423-428
  • View abstract

    Summary

    The association of elevated hematocrit and fibrinogen levels with increased stroke risk has led to continued interest in hemorheologic factors and their role in the development of vascular disease and acute stroke. Viscosity increases logarithmically at the lowest shear rates and this effect is magnified at higher hematocrit levels. Fibrinogen may play a causal role in ischemic stroke through several mechanisms. The most prominent of these is its essential role in thrombosis, both as the substrate for fibrin clot formation and as a facilitator of platelet aggregation. The risk of stroke in sickle cell disease (SCD) varies according to genotype and is highest in patients with homozygous SS. Plasma hyperviscosity syndromes are treated by plasmapheresis (plasma exchange) to remove the paraproteins and thereby reduce hyperviscosity and hypervolemia. Newer techniques of cell centrifugation, plasma separation, and filtration may also be useful.
  • 59 - SCLERODERMA
    pp 429-432
  • View abstract

    Summary

    This chapter reviews the role of calcium and magnesium in causing and ameliorating brain ischemia, respectively. Three different effects of hypercalcemia are posited to contribute to the development and severity of brain ischemia: (i) hypercalcemia stimulates vascular smooth muscle causing vasoconstriction; (ii) increased calcium concentrations enhance platelet aggregation and activate the body's intrinsic coagulation system; and (iii) calcium entry into cells, a process enhanced by an elevated extracellular-to-intracellular calcium ion gradient, causes cytotoxic effects that promote cell death and brain infarction. The rationale for the use of calcium antagonists in the prevention or treatment of secondary brain ischemia was based on the assumption that these drugs reduce the frequency of vasospasm by counteracting the influx of calcium into vascular smooth muscle cells. There is some benefit from the use of the dihydropyridine CCA nimodipine for improving outcome after aneurysmal subarachnoid hemorrhage.
  • 60 - CERVICO-CEPHALIC ARTERIAL DISSECTIONS
    pp 433-454
  • View abstract

    Summary

    This chapter explores the complex relationship of stroke with the usage of abusive drugs. Opiates, amphetamines, cocaine, D-lysergic acid diethylamide (LSD), marijuana, sedatives, ethanol, tobacco and phencyclidine are investigated. Hemorrhagic stroke in heroin users may be a consequence of hepatitis with liver failure and deranged clotting or of heroin nephropathy with uremia or malignant hypertension. Amphetamine-induced cerebral vasculitis has also caused ischemic stroke. Cocaine increases the likelihood of vasospasm after aneurysm rupture. D-lysergic acid diethylamide (LSD), phencyclidine and marijuana users reports symptoms of stroke. Barbiturates, benzodiazepines, and other sedative drugs can cause cerebral infarction whereas hemorrhagic stroke has not otherwise been reported. Aneurysmal rupture occurred during orgasm following amyl nitrite inhalation. Multiple mechanisms probably explain the complex association of ethanol and stroke. Smoking is a major risk factor for coronary artery and peripheral vascular disease. Tobacco increases the risk for both ischemic and hemorrhagic stroke.
  • 61 - CEREBRAL AMYLOID ANGIOPATHIES
    pp 455-464
  • View abstract

    Summary

    Paraneoplastic strokes show a clinical picture different from those observed in stroke patients without cancer. Diffuse and progressive encephalopathy, either isolated or accompanied by focal neurological manifestations, is usual in disseminated intravascular coagulation, nonbacterial thrombotic endocarditis, and paraneoplastic vasculitis. The clinical presentation of paraneoplastic cerebral venous thrombosis includes severe, diffuse, and progressive headache due to intracranial hypertension, partial or generalized seizures, transient ischemia or cerebral infarct of venous origin, and progressive ischemic encephalopathy. The diagnostic process for paraneoplastic strokes is different from standard procedures for other types of stroke. Cerebral computed tomography (CT) and magnetic resonance imaging (MRI) can be normal in ischemic events caused by disseminated intravascular coagulation, or may show multiple images in several vascular territories in nonbacterial thrombotic endocarditis and vasculitis. Heparin may be effective in preventing cerebral infarction among patients with nonbacterial thrombotic endocarditis and does not increase the risk of hemorrhagic complications.
  • 62 - MOYA-MOYA SYNDROME
    pp 465-478
  • View abstract

    Summary

    Kohlmeier-Degos' disease (KDD) is more common in white boys and men with age of onset ranging between 3 weeks of age to 67 years old, most commonly occurring in the second and third decades. The etiology and pathogenesis, although still unknown, is speculated to be immunologic dysfunction, coagulation or fibrinolysis abnormalities, or possible viral infections. Any organ system may be involved; the clinical manifestations are a result of multifocal infarctions. Laparoscopic examination, however, shows lesions similar in appearance to the skin lesions that may involve multiple areas of the small intestine. These lesions are secondary to infarction and have also been found in the esophagus, duodenum, stomach, colon, and rectum. Ischemia and infarction are key pathophysiological mechanisms of the lesions seen. Therapies that have been used include antiplatelets, anticoagulants, immunosuppressive agents, plasma exchange, and nicotine patches. Surgery is indicated in cases of intestinal perforation.
  • 63 - DILATATIVE ARTERIOPATHY (DOLICHOECTASIA)
    pp 479-482
  • View abstract

    Summary

    This chapter highlights the clinical, pathophysiologic, and biochemical links between inflammatory bowel disease (IBD) and ischemic stroke. Deep venous thrombosis (DVT) and pulmonary embolism (PE) account for the overwhelming majority of thrombotic events in patients with IBD. Descriptions of IBD-associated central nervous system (CNS) vasculitis are based on angiography showing segmental narrowing in the small to medium-sized arteries. Of the many IBD-related hypercoagulable state manifestations, an estimated 10 percentage are ischemic strokes. Hyperhomocysteinemia has recently emerged as an independent risk factor for stroke; homocysteine is a sulfur-containing amino acid formed during the demethylation of the amino acid methionine. The mechanism for ischemic strokes in IBD patients is multifactorial, but likely secondary to an abnormal coagulability profile and, in rare situations, cerebral vasculitis. Better management of IBD flares, improved compliance with newer IBD medications, and quicker surgical interventions might lead to better outcomes and stroke risk reduction.
  • 64 - PARADOXICAL EMBOLISM AND STROKE
    pp 483-490
  • View abstract

    Summary

    Sweet's syndrome (SS), also referred to as acute febrile neutrophilic dermatosis, is characterized by a constellation of symptoms and findings: fever, neutrophilia, erythematous and tender skin lesions that typically show an upper dermal infiltrate of mature neutrophils, and prompt improvement of both symptoms and lesions after the initiation of treatment with systemic corticosteroids. Many instances are related to lympho proliferative disorders such as acute and chronic leukemia, acute and chronic lymphatic leukemia, hairy cell leukemia, polycythemia vera, non-Hodgkin's lymphoma, Hodgkin's lymphoma, and other diseases of the hematopoietic system. Encephalitis and meningitis were the most common neurological manifestations. An elevated erythrocyte sedimentation rate (ESR) and peripheral leukocytosis with neutrophilia are the most consistent laboratory findings in SS. The standard therapy is administration of prednisone or prednisolone at an initial dose of 0.5-1.5 mg/kg body weight with a subsequent slow reduction over 2-4 weeks.
  • 65 - FIBROMUSCULAR DYSPLASIA
    pp 491-496
  • View abstract

    Summary

    Minimal change disease (MCD) is the most common cause of nephrotic syndrome in children, although it is not uncommon among adults. Endothelial cell injury, platelet hyperreactivity and hyperaggregability secondary to increased activity of adenosine biphosphate lead to thrombosis. Microalbuminuria is an index of generalized vascular endothelial dysfunction, especially in hypertension and diabetes and a well-recognized risk factor for stroke in men and women, independent of other vascular risk factors and regardless of stroke mechanism. Hyperlipidemia may also contribute to the increased risk for thrombosis seen in nephrotic syndrome. Cerebral arterial infarction is an uncommon yet treatable cause of stroke in patients with nephrotic syndrome, predominantly in patients with membranous glomerulo nephropathy (MGN) followed by focal segmental glomerulosclerosis and immunoglobulin A (IgA) nephropathy, and rarely minimal change nephropathy. Magnetic resonance angiography and magnetic resonance venography are helpful to confirm the diagnosis, and dye contrast catheter cerebral angiography is rarely necessary.
  • 66 - CEREBRAL VENOUS SINUS THROMBOSIS
    pp 497-504
  • View abstract

    Summary

    The epidermal nevus syndrome refers to the association of any epidermal nevus with extracutaneous abnormalities. The most common extracutaneous abnormalities are neurologic, skeletal, and ocular, although other organs may also be involved. Widespread use of magnetic resonance imaging (MRI) has resulted in an increasing appreciation of the role of cortical malformations in patients with neurological symptoms. Strokes and vascular abnormalities have been reported in patients with the epidermal nevus syndrome. Arteriovenous malformations and leptomeningeal angiomas have been found in some patients with the epidermal nevus syndrome. Generally, the diagnosis of an epidermal nevus is not in doubt. If there are any doubts, a skin biopsy should be obtained. Treatment of the nevus with dermabrasion, diathermy, laser treatment, and cryotherapy is associated with a fairly high risk of recurrence of the nevus. Focal resection of the epidermal nevus before puberty is advised because of the increased risk of tumor development.
  • 67 - REVERSIBLE CEREBRAL VASOCONSTRICTION SYNDROMES
    pp 505-514
  • View abstract

    Summary

    Sneddon's syndrome (SS) refers to an infrequent disorder combining skin and ischemic cerebral lesions in patients without a recognizable connective tissue or inflammatory or chronic infectious disease. As a rule, the transient ischemic attacks and strokes are multiple and recurrent in the same or different vascular territories. Both cortical and subcortical areas in the anterior and posterior circulation can be affected, but the most common lesions leading to progressive clinical disability are white matter abnormalities and lacunar infarcts. A large retrospective study on the anti-phospholipid antibody syndrome demonstrated that treatment with high-intensity warfarin (producing an international normalized ratio [INR] of >3) with or without low-dose aspirin (75 mg/day) is significantly more effective than treatment with low-intensity warfarin (producing an INR of <3) with or without treatment with low-dose aspirin or treatment with aspirin alone in preventing further thrombotic events.
  • 68 - ECLAMPSIA AND STROKE DURING PREGNANCY AND THE PUERPERIUM
    pp 515-528
  • View abstract

    Summary

    This chapter, reviews mitochondrial and other selective metabolic causes of stroke. In MELAS, despite the microangiopathic findings in the brain and muscles, the stroke-like episodes are more likely attributed to mitochondrial and metabolic dysfunction in neural tissue and glia rather than to ischemic vascular pathology. Kearns Sayre syndrome (KSS) is a mitochondrial disorder caused by large heteroplasmic deletions in mtDNA. Brain infarction, presumably secondary to cardioembolic sources, may occur. Hyperhomocysteinemia is a clinical syndrome caused by several enzyme deficiencies in methionine metabolism. Patients with homocystinuria have markedly elevated plasma homocysteine concentrations. Most patients present with peripheral venous thrombosis, including pulmonary embolism. Stroke, peripheral arterial occlusions, or myocardial infarction can be the initial presentation. The increased tendency for thrombosis usually presents as an ischemic stroke. Metabolic stroke due to hypoxemia and vascular insufficiency may occur in methylmalonic acidemia. Metabolic causes of stroke are quite heterogeneous.
  • 69 - MIGRAINE AND MIGRAINE-LIKE CONDITIONS
    pp 529-532
  • View abstract

    Summary

    This chapter reviews rare stroke occurrences associated with specific bone disorders. The disorders have been divided into subgroups based on body bone pathology, specific skull diseases, and periodontal diseases. The phenomenon of fibrocartilaginous embolism (FE) is well documented in the veterinary literature. Multifocal ischemic encephalomyelopathy associated with fibrocartilaginous emboli was first described in a lamb by Jeffrey and Weels. Osteopetrosis (OP) is a rare, hereditary metabolic disorder of unknown etiology, characterized by an abnormal accumulation of bone mass, probably caused by diminished bone resorption. Cerebrovascular disorders of these patients have a mainly mechanical origin. Craniosynostosis may be associated with decreased cerebral blood flow as a result of the constriction of the brain because of the prematurely fused sutures. There is a relationship between premature arteriopathy with stroke and Camurati-Engelmann disease (CED). The association between periodontal disease and stroke has several possible pathophysiologic links.
  • 70 - INTRAVASCULAR LYMPHOMA
    pp 533-538
  • View abstract

    Summary

    Scleroderma (progressive systemic sclerosis) is a multisystem connective tissue disorder characterized by inflammation, fibrosis, and vasculopathy of affected tissues. CNS vasculitis, segmental vasospasm, and cerebrovascular calcifications may all play a role in causing strokes in patients with scleroderma. CNS vasculitis has been diagnosed in several patients with scleroderma and has been posited to cause strokes. Cerebral infarction in scleroderma patients in the absence of other plausible, causative factors should prompt an aggressive workup for vasculitis including angiography. Results of cerebral angiography in several patients thought to have vasculitis are consistent with the diagnosis of vasoconstriction. Arteriography revealed segmental, often smoothly contoured, narrowing of arteries of multiple sizes (small, medium, and large) in both the anterior and posterior circulations. Whether vascular calcium deposits were responsible for the patients' cerebrovascular symptoms is speculative. Scleroderma patients with cerebrovascular disease must take into consideration the potential causes of stroke.
  • 71 - OTHER CONDITIONS
    pp 539-544
  • View abstract

    Summary

    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.

Page 3 of 3

Metrics

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Book summary page views

Total views: 0 *
Loading metrics...

* Views captured on Cambridge Core between #date#. This data will be updated every 24 hours.

Usage data cannot currently be displayed.