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striking relief of the headache and polymyalgic symptoms within days and sometimes within hours and also prevents blindness The medication must be given in very gradually diminishing doses for at least several months or longer, guided by the symptoms and the sedimentation rate The latter begins to drop within days but seldom falls below 25 mm/h Intracranial Granulomatous Arteritis Scattered examples of a small-vessel giant-cell arteritis of undetermined etiology in which only brain vessels are affected have come to medical attention over the years The clinical state has taken diverse forms, sometimes presenting as a low-grade, nonfebrile meningitis with sterile CSF, followed by infarction of one or several parts of the cerebrum or cerebellum In other cases it has masqueraded as a cerebral tumor, evolving over a period of weeks, or as a viral encephalitis or an unusual dementia Headaches (variable in our experience but sometimes severe), focal cerebral or cerebellar signs of gradual (seldom stroke-like) evolution, confusion with memory loss, pleocytosis and elevated CSF protein, and papilledema as a result of increased intracranial pressure (in about half the cases) constitute the most frequently encountered syndrome The symptoms usually persist for several months In contrast to temporal arteritis, the sedimentation rate is generally normal or only slightly elevated In only about half the patients can the diagnosis be made by angiography, which demonstrates an irregular narrowing and in some cases blunt ending of small cerebral arteries (Fig 34-31) CT scanning and MRI show multiple irregular white matter and cortical changes and small cortical lesions; often these cannot be differentiated from a tumor or infectious process Occasionally the white matter abnormalities become con uent and the radiologic appearance simulates Binswanger disease or hypertensive encephalopathy The diagnosis is made most often by a brain biopsy, which includes a sample of the meninges, but even with tissue sampling, only about half of suspected cases show the typical histopathologic changes; often, however, patients with normal angi-.

Figure 34-31 Granulomatous angiitis of the brain Carotid angiogram, lateral projection, demonstrating numerous areas of irregular narrowing (arrows) and, in some areas, contiguous slight dilation ( beading ), particularly in the anterior cerebral artery

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ograms will have the typical ndings on biopsy As pointed out by Alrawi and colleagues, many patients prove to have an alternative condition, mainly an infectious encephalitis and brain or intravascular lymphoma, abscess, or Creutzfeldt-Jakob disease Tissue excised during an operation (or brain biopsy) for a suspected brain tumor, lymphoma, or white matter disease has revealed the characteristic vasculitis in some cases; in others the diagnosis has been made only at autopsy, the ndings coming as a distinct surprise The affected vessels are in the 100- to 500-mm range and are surrounded and in ltrated by lymphocytes, plasma cells, and other mononuclear cells; giant cells are distributed in small numbers in the media, adventitia, or perivascular connective tissue Infarction of brain tissue can be traced to thrombosis The meninges are variably in ltrated with in ammatory cells Sometimes only a part of the brain has been affected in one of our cases the cerebellum, in another, one frontal lobe and the opposite parietal lobe Among the most important considerations is the cerebral arteritis caused by varicella zoster virus, which can simulate in radiographic appearance granulomatous arteritis and giant cell arteritis (page 643) The viral form of arteritis follows or evolves in association with herpes zoster ophthalmicus On occasion, intravascular lymphoma may present a similar picture The clinical and radiologic appearance of brain arteritis also raises the question of sarcoidosis, which is sometimes limited to the nervous system, of brain lymphoma or intravascular lymphoma, of CADASIL, multiple sclerosis, or of the polyarteritis (allergic granulomatous angiitis) described by Churg and Strauss Unlike some of these diseases, however, the lungs and other organs are spared; there is no eosinophilia, increase in sedimentation rate or antineutrophil cytoplasmic antibodies (ANCA), or anemia As mentioned, brain lymphoma of the intravascular type and multiple sclerosis are diagnostic considerations from both a clinical and a radiologic perspective Some patients with isolated central nervous system angiitis (those presenting as an aseptic meningitis with multiple infarcts) have responded to corticosteroid and cyclophosphamide therapy (Moore) Takayasu Disease (Aortic Branch Disease, Occlusive Thromboaortopathy) This is a nonspeci c arteritis involving mainly the aorta and the large arteries arising from its arch It is similar in many ways to giant-cell arteritis except for its propensity to involve the proximal rather than the distal branches of the aorta Most of the patients have been young Asian women, but there are scattered reports of similar cases from the United States, Latin America, and eastern Europe The exact etiology has never been ascertained, but an autoimmune mechanism is suspected Constitutional symptoms such as malaise, fever, anorexia, weight loss, and night sweats usually introduce the disease The erythrocyte sedimentation rate is elevated in the early and active stages Later there is evidence of occlusion of the innominate, subclavian, carotid, vertebral, and other arteries The affected arteries no longer pulsate, hence the descriptive term pulseless disease However, it should be noted that in non-Asian individuals, the disease is usually due to atherosclerosis When renal arteries are involved, hypertension may result, and there may be coronary occlusion, which is often fatal Involvement of the pulmonary artery may lead to pulmonary hypertension Coolness of the hands and weak radial pulses are common indicators of the disease and headaches are frequent Blurring of vision, especially upon physical activity, dizziness, and hemiparetic and hemisensory syndromes are the usual neurologic manifestations (Lupi-Herrera et al) Several au-.

Now that I ve shown you how easy it is to get up and running quickly with the Pattern Wizard, we will look at the Information Consumer Pattern Although this is the first option under BI Patterns with Pattern Wizard, it should be the last one that you try because it is the most involved Please refer to Figure 2-1 for the initial screen with the Information Consumer Pattern option The Information Consumer Pattern template offers you the choice to increase the functionality and format of each of the Web queries twofold over the Small Web Templates previously discussed This template is a series of steps that walks you through the entire WAD 0ANALYSIS_PATTERN template, enabling you to turn off or leave on any of the numerous parameters for the query Step 1, illustrated next, shows immediately that this is very different from the other template wizards

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