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  • Cited by 19
Cambridge University Press
Online publication date:
January 2010
Print publication year:
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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.


Highly Commended at the BMA Book Awards 2009


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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Page 1 of 3

    pp 41-46
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    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.
    pp 53-58
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    Temporal (giant cell) arteritis is a systemic disease, involving various medium-sized and larger arteries, that occurs mostly in elderly patients. Blindness due to ischemic optic neuropathy is probably the most common and most feared sinister manifestation of the disease, but stroke is the leading cause of death in patients with temporal arteritis. Noninvasive angiography using computed tomography (CT) or magnetic resonance imaging (MRI) may reveal sites of vascular stenoses. A typical finding is smoothly tapered stenotic lesions different from the abrupt, irregular stenoses of atherosclerotic disease. These modalities may be helpful in assessing the extent of disease or potentially to aid diagnosis especially in biopsy negative cases. Fludeoxyglucose-positron emission tomography (FD-PET) scanning may reveal uptake in the larger thoracic vessels including aorta, subclavian, and carotid arteries. The mainstay of treatment is corticosteroids, although there is much debate about the optimal dose and use of steroid sparing immunosuppressives.
    pp 59-66
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    Varicella zoster virus infections of the central nervous system (CNS) develop preferentially in immunocompromised individuals, especially those affected by HIV and cancer. It is unclear whether treatment of herpes zoster with antiviral agents prevents the development of subsequent cerebral vasculopathy and stroke. Herpes zoster-related cerebral vasculopathy is well-documented as herpesvirus nucleocapsids have been detected by electron microscopy. Primary granulomatous angiitis of the nervous system (PACNS) is a disease in which CNS is the sole or dominant target organ of a vasculitic process, affecting the small and medium leptomeningeal and cortical arteries and, less frequently, the veins and venules. Clinical studies have reported an increased prevalence of cytomegalovirus (CMV) and herpes simplex virus (HSV) infections among individuals with accelerated atherosclerosis in the extracranial carotid arteries. In addition, histopathological studies have detected CMV and HSV particles within atherosclerotic vessels, and infection with HSV-induced atherosclerosis in avian models.
    pp 67-74
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    In Takayasu disease, the aortic arch, with its main arterial trunks and the descending aorta, as well as renal arteries, is the main site of inflammation. Takayasu disease is thought to begin clinically with symptoms of systemic inflammation or with eye symptoms. Cerebral vascular disease is usually a consequence of severe hypertension, or carotid or brachiocephalic obstruction. Recently, it has been emphasized that use of Doppler ultrasound, computed tomography angiography, and positron emission tomography (PET) scan, magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) in combination, may facilitate the detection of Takayasu disease activity at a more treatable stage. Concerning the treatment and management of Takayasu disease patients with stroke, one has to consider the treatment of Takayasu disease and stroke separately. Subclavian steal syndrome or moderate carotid stenosis, as well as renal artery stenosis may be an indication for angioplasty and/or stenting.
    pp 75-80
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    Bürger's disease or thromboangiitis obliterans (TAO) is a nonatherosclerotic segmental inflammatory obliterative vascular disease that affects medium- and small-sized arteries as well as superficial veins. Bürger's disease manifested in the brain has the following morphologic characteristics: arterial occlusions caused by thrombosis in small arteries without arteriosclerosis, and spatial predilection for the cerebral surface in the watershed region between the middle cerebral, anterior, and posterior cerebral arteries. TAO is characterized by claudication or ischemia of both legs and less so of the arms. The disease begins distally and progresses more proximally. The diagnosis of TAO requires the exclusion of an embolic source, autoimmune disease, diabetes, and hyperlipidemia. Arteriography should show the distal involvement with normal arterial lumen proximal to the popliteal or distal brachial level and the absence of atheromatous changes in the large vessels. Abstinence from tobacco will likely halt disease progression and sometimes result in regression of vascular changes.
    pp 81-86
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    Neurosyphilis begins with invasion of the central nervous system (CNS) by spirochetes during the period of spirochete dissemination from the primary lesion (called secondary syphilis). The pathology of meningovascular syphilis has two major components. The cause of most cerebrovascular disease is syphilitic endarteritis, usually involving medium-to-large meningeal arteries, called Heubner's endarteritis. Brain CT or magnetic resonance imaging of patients with meningovascular syphilis and stroke typically show abnormalities consistent with ischemic lesions, which may be multiple. Conventional angiography or magnetic resonance angiography typically shows evidence of arteritis with concentric narrowing of large vessels and often focal narrowing and occasionally dilatation of smaller arteries. Syphilis of the spinal cord is a clinical rarity and usually accompanies other forms of cerebral syphilitic involvement. Ceftriaxone is currently the alternative treatment of choice in the few patients who cannot be desensitized to penicillin.
    pp 87-92
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    Tuberculous meningitis (TBM) is still a crippling disease with a high degree of morbidity and mortality. One of the most severe complications of TBM is stroke resulting from vascular involvement. In HIV-infected individuals with TBM, the immune response to the tuberculous bacilli is altered; therefore, pathological features are very different from those seen in patients with relatively normal cell-mediated immunity (CMI). The brains of such individuals showed minimal inflammatory response with parenchymal infarcts and vasculitis, not only in the basal ganglia but in the cortical parenchyma as well. Computed tomography (CT) or magnetic resonance imaging (MRI) of the head may reveal intense basal enhancement after intravenous contrast administration, communicating or noncommunicating hydrocephalus, cerebral infarcts, parenchymatous tuberculomas, or a combination of two or more of these features. Besides chemotherapy, patients of TBM with vasculitis and infarcts should be given corticosteroids in a dose of 1 mg/kg/day, tapering this over 4-6 weeks.
    pp 93-100
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    This chapter investigates the complex relationship of stroke with fungal pathogens like aspergillus, mucor, candida, cryptococcus and other fungi. Among fungal pathogens, aspergillus is most likely to present as a stroke or stroke-like syndrome. Pathologically there were multiple areas of cerebral infarction with thrombosis due to Aspergillus invasion of arteries. Like Aspergillus, Mucor species are angioinvasive and can cause stroke through, bland infarction, and vascular thrombosis. Candida is not an angioinvasive pathogen, and reports of stroke after candida infection are distinctly uncommon. Like candida species, cryptococcus is not angioinvasive. Cryptococcus neoformans is an encapsulated yeast. A variety of less common fungi that can rarely infect immunocompromised patients include fusarium (a septate mold), Trichosporon (a pathogenic yeast), paecilomyces, pseudallescheria, Scopulariopsis, and the endemic fungi (coccidioides immitis, histoplasma capsulatum). Reports of central nervous system (CNS) involvement or stroke like syndromes are limited with these less common fungi.
    pp 101-108
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    Cysticercosis is caused by infection with the larval stage ofTaenia solium, the pork tapeworm. A brief description of the many changes that cysticerci induce in the central nervous system (CNS) is necessary to understand the pathogenesis of neurocysticercosis (NCC) related stroke. Lacunar infarctions occur as the result of inflammatory occlusion of small penetrating branches of the middle cerebral artery (MCA). Another cerebrovascular complication of NCC is hemorrhagic stroke. Stroke is common among patients with subarachnoid NCC, but it is seldom observed in other forms of the disease. CT and MRI show the infarction as well as the characteristic findings of subarachnoid NCC, including abnormal enhancement of leptomeninges, hydrocephalus, and cystic lesions located at the sylvian fissure or basal cisterns. Introduction of cysticidal drugs (albendazole and praziquantel) have greatly improved the prognosis of NCC by destroying intracranial cysts and improving the neurological manifestations in most patients with parenchymal NCC.
    pp 109-114
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    Lyme disease, the multisystem infectious disease caused by the tick-borne spirochete Borrelia burgdorferi, readily invades the central nervous system (CNS) and, in up to 15% of patients, causes symptomatic meningitis or involvement of the cranial or spinal nerves. The clinical evidence supporting an association between B. burgdorferi infection and cerebral vasculitis or stroke is tenuous at best. Unfortunately, parenchymal brain disease has not been reported in any animal model. Peripheral nerve disease occurs fairly commonly both in infected patients and in experimentally infected rhesus macaque monkeys. Although in both humans and monkeys this is a patchy multifocal disease (mononeuritis multiplex), with perivascular inflammatory infiltrates evident in biopsied nerves, in neither has there ever been evidence of a true vasculitis or significant vasculopathy. Thus, although neurosyphilis has been known for many years to cause vascular inflammation and damage, to date there is little proof that this occurs in Lyme disease.
    pp 115-122
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    Behçet's disease (BD) is a multisystemic inflammatory disorder of unknown etiology, and neurologic involvement is one of the major clinical features. When the clinicopathological and neuroradiological findings are combined, two different patterns of central nervous system (CNS) involvement in BD can be established: parenchymal and neurovascular. Neurologic involvement is one of the most devastating manifestations of BD. This involvement may occur primarily within the nervous parenchyma (n-BD) or secondarily in the cerebral vascular system (vasculo[angio]-BD). Thrombosis of cerebral large veins and sinuses is the most common feature of vasculo-BD, although thrombosis of the vena cava and portal vein may also occur in one third of these patients. BD is usually included among the systemic vasculitides but documented cerebral arteritis is extremely rare and even a debatable mechanism for CNS involvement. Cerebrovascular complications of BD are rarer than parenchymal involvement of the CNS and aseptic meningitis.
    pp 123-130
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    This chapter investigates sarcoidosis-associated stroke syndromes. Sarcoidosis is often referred to as a "disease of exclusion". The definitive diagnosis of sarcoidosis requires histopathologic demonstration of noncaseating epithelioid granulomas that are not due to infection or malignancy. Patients can be classified as having possible, probable, or definite neurosarcoidosis based on the certainty of the diagnosis of multisystem sarcoidosis, the pattern of neurological disease, and the response to therapy. Corticosteroids are the mainstay of treatment for patients with symptomatic neurosarcoidosis; however, the severity and chronicity of this disease in some patients often leads to reluctance in subjecting patients to the long-term sequelae of corticosteroid treatment. Nonetheless, it is reasonable to decrease sarcoidosis-associated inflammation to decrease stroke risk. Anecdotal evidence suggests that concurrent use of corticosteroids and adjunctive agents is a reasonable approach. Antiplatelet agents has been used as a stroke-preventive strategy but have avoided thrombolytic interventions.
    pp 131-134
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    Neurological signs and symptoms are common in Kawasaki disease and most often include aseptic meningitis, encephalopathy, and sensorineural hearing loss. Diagnosis is clinically based on evolving diagnostic criteria. Although stroke in patients with Kawasaki disease has not been methodically studied, several reports exist of hemiparesis in children diagnosed with Kawasaki disease, some of which include angiographically diagnosed vascular lesions. Early attempts to treat Kawasaki disease focused on the use of aspirin, glucocorticoids, and immune modulators such as azathioprine. Review of the current literature suggests that stroke, and specifically asymptomatic brain infarction, may be more common than now recognized, especially in patients with coronary artery abnormalities. Thorough clinical, neuroradiological, and neuropsychological assessments of these patients are indicated to gain a full appreciation of the nervous system involvement and the long-term neurodevelopmental sequelae resulting from this disease.
    pp 135-138
  • View abstract


    Chagas' cardiomyopathy is the most frequent and severe clinical manifestation of chronic Chagas' disease, and is associated with poor prognosis and high mortality rate. Cardiac arrhythmias may cause palpitations, lightheadedness, dizziness, and syncope. Autonomic dysfunction results in marked heart rate abnormalities, especially bradycardia. Sudden death is an occasional complication that may be precipitated by exercise and can be explained by ventricular tachycardia or fibrillation or complete heart block. Mural thrombi form in cardiac chambers and may result in systemic emboli. Stroke is the most important complication of embolism in Chagas' cardiomyopathy. The most commonly used diagnostic test is based on complement fixation, immunofluorescence, or enzyme-linked immunofluorescence assays. Chagas' disease can be diagnosed with greater sensitivity by the detection of Trypanosoma cruzi-specific sequence of DNA. Chagas' disease patients with acute ischemic stroke may also benefit from recombinant tissue plasminogen activator (rtPA).
    pp 139-144
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    Stroke is uncommon in HIV-positive individuals. There is mounting evidence to indicate that HIV infection is associated with an increased risk for both ischemic and hemorrhagic stroke. A number of mechanisms, discussed in this chapter, have been proposed for this increased risk including prothrombotic states, other secondary causes such as cerebral infections and cardioembolic causes, as well as a primary HIV associated vasculopathy. The most common mechanisms cited for stroke development in AIDS patients are: (1) hypercoagulable or prothrombotic states; (2) other secondary causes such as brain embolism and cerebral infections; and (3) direct primary HIV-associated vasculopathies. Two forms of vasculopathy have been described that have been considered to be HIV-specific: small vessel HIV-associated vasculopathy which is an asymptomatic vasculopathy, and large- and medium-vessel HIV-associated vasculopathy which involves both extracranial arteries as well as medium-sized intracranial vessels.

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