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Noninfectious In ammatory Diseases of Cranial Arteries
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Included under this heading is a diverse group of arteritides that have little in common except their tendency to involve the cerebral vasculature One subgroup includes the giant-cell arteritides extracranial (temporal) arteritis; granulomatous arteritis of the brain; and aortic branch arteritis, one form of which is known as Takayasu disease All are discussed on the following pages A second group includes polyarteritis nodosa, the ChurgStrauss type of arteritis, Wegener granulomatosis, systemic lupus erythematosus, Behcet disease, post-zoster arteritis, and AIDS-re lated arteritis Immunologic studies have shown that in most of these diseases there is an abnormal deposit of complement- xing immune complex on the endothelium, leading to in ammation, vascular occlusion, or rupture with hemorrhage The initial event is thought in some cases to be evoked by a virus, bacterium, or drug It is postulated by some immunologists that in the granulomatous arteritides, a different mechanism is operative that an
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exogenous antigen induces antibodies that attach to the primary target (the vessel wall) as immune complexes, damage it, and attract lymphocytes and mononuclear cells The giant cells form around remnants of the vessel wall (elastic tissue, etc) An acute necrotizing cerebral angiitis which may be idiopathic, sometimes complicates ulcerative colitis, and responds to treatment with prednisone and cyclophosphamide may also belong in this category The special case of intravascular lymphoma, which simulates a cerebral vasculitis, is discussed in Chap 31 Temporal Arteritis (Giant-Cell Arteritis, Cranial Arteritis; See also page 159) In this disease, not uncommon among elderly persons, arteries of the external carotid system, particularly the temporal branches, are the sites of a subacute granulomatous in ammatory exudate consisting of lymphocytes and other mononuclear cells, neutrophilic leukocytes, and giant cells The most severely affected parts of the artery usually become thrombosed The sedimentation rate is characteristically elevated above 80 mm/h and sometimes exceeds 120 mm/h, but a small number of cases occur with values below 50 mm/h Headache or head pain is the chief complaint, and there may be severe pain, aching, and stiffness in the proximal muscles of the limbs associated with the markedly elevated sedimentation rate Thus the clinical picture overlaps that of polymyalgia rheumatica as discussed in Chap 10 Other less frequent systemic manifestations include fever, anorexia and loss of weight, malaise, anemia, and a mild leukocytosis Instances of dementia, depression, and other neurologic illnesses that have been described in the literature in patients with temporal arteritis seem to us coincidental Occlusion of branches of the ophthalmic artery, resulting in blindness in one or both eyes, is the main complication, occurring in over 25 percent of patients In the most extreme form, the optic nerve head can be seen to be infarcted, with papilledema and visual loss This is a form of anterior ischemic optic neuropathy discussed in Chap 10 In a few cases blindness is preceded by transient visual loss simulating a TIA Occasionally the arteries of the oculomotor nerves are also involved, causing an ophthalmoplegia Rarely, an arteritis of the aorta and its major branches including the carotid, subclavian, coronary, and femoral arteries is found at postmortem examination Signi cant in ammatory involvement of intracranial arteries from temporal arteritis is uncommon, perhaps because of a relative lack of elastic tissue, but in a few cases strokes have occurred on the basis of occlusion of the internal carotid or vertebral arteries The diagnosis should be suspected in elderly patients who develop severe, persistent headache and elevation of the sedimentation rate; it depends on nding a tender, thrombosed, or thickened cranial artery and demonstration of the lesion in a biopsy The procedure is innocuous and the diagnosis may require that both sides be sampled because of the patchy distribution of granulomatous lesions Schmidt and colleagues have reported that the diagnosis can often be made with duplex ultrasonography In 22 of 30 cases, a dark halo, probably re ecting edema, surrounded the affected temporal artery; 6 cases showed either occlusion or stenosis of the artery; there were no false-positive tests A considerable length of the temporal artery can be insonated by this technique, a particularly useful feature in a process that affects the vessel segmentally From our limited experience, these ndings have proven dif cult to detect The arteritic changes may also be revealed by arteriography of the external carotid arteries The administration of prednisone, 50 to 75 mg/day, provides
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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-
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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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